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DeVries & Pont has been appointed by Golf de Fontainebleau DeVries & Pont has been appointed as consulting golf course architects at the highly regarded Golf de Fontainebleau club in France oak and pine trees on the outskirts of the town of Fontainebleau Julien Chantepie laid out the course in 1909 with a substantial redesign completed by Tom Simpson in 1920 “We are honoured to have been asked to assist Golf de Fontainebleau,” says Frank Pont “The course sits on a truly beautiful and special site we shall be undertaking a full audit of the bunkering.” I hadn’t seen Fontainebleau since I played a Legends Tour tournament there about 10 years ago Florence and Dominique Chantepie's new wine bar will open at 40 Archdale Street this spring In France, a Bistro A Vin is a place that specializes in its own house wine purchasing directly from a vineyard and creating a unique blend Dominique and Florence Chantepie won't be able to recreate that exact sort of wine bar here in Charleston but the husband and wife team will be specializing in French wines opening soon at 40 Archdale Street in downtown Charleston "But the idea is the same," says Dominique "We'll be able to bring these small vineyards in here." Dominique says French wine is not just Bordeaux The French expatriates have been operating Café Framboise next door for three years The patisserie specializes in freshly made pastries like croissants and macarons but also serves salads and great sandwiches a regular at the cafe who lives around the corner It's those sort of neighbors that the Chantepies expect to haunt their new wine bar which shares an outdoor terrace with the cafe The building most recently housed an olive oil store and when it came available they decided it would be perfect for a wine bar Inside, exposed brick walls date back to the mid-1800s. According to an archaeological study performed before Majestic Square was developed 40 Archdale Street "has been used as a grocery or pool hall for virtually all of its nineteenth and early twentieth century history."  says the wine bar will also have coffee and tea along with plated desserts specially created next door for the wine bar and we'll have special wines that go well with dessert: Banyul they will have 40 French wines with 20 available by the glass in the range of $9-$15 on par with what Bin 152 charges nearby on King Street Florence says they will serve French cheeses some from local vendors and some imported directly from France and hope to turn locals on to some wines they might not be familiar with "There's a big trend to organic wines," says Dominique "We want to be specialized in those kinds of wines The techniques are good and the wines are good They expect to be ready to open sometime in March Follow Stephanie Barna on Twitter @stefbarna Take a seat at the bar and prepare yourself for a glass of bubbles with cassis or framboise It's the perfect way to start an evening at Bistr… News tips/online questions: newstips@postandcourier.com Delivery/subscription questions: subserve@postandcourier.com Your browser is out of date and potentially vulnerable to security risks.We recommend switching to one of the following browsers: Metrics details Skeletal dysplasia with multiple dislocations are severe disorders characterized by dislocations of large joints and short stature The majority of them have been linked to pathogenic variants in genes encoding glycosyltransferases sulfotransferases or epimerases required for glycosaminoglycan synthesis we identify homozygous mutations in SLC10A7 in six individuals with skeletal dysplasia with multiple dislocations and amelogenesis imperfecta SLC10A7 encodes a 10-transmembrane-domain transporter located at the plasma membrane Functional studies in vitro demonstrate that SLC10A7 mutations reduce SLC10A7 protein expression growth plate disorganization and tooth enamel anomalies we identify decreased heparan sulfate levels in Slc10a7−/− mouse cartilage and patient fibroblasts we find an abnormal N-glycoprotein electrophoretic profile in patient blood samples our findings support the involvement of SLC10A7 in glycosaminoglycan synthesis and specifically in skeletal development These findings suggest that GAG synthesis is more complex than previously described and suggests that there are a number of partners of unknown function still to be identified Using a deficient mouse model and patient fibroblasts we demonstrate that SLC10A7 deficiency disrupts GAG biosynthesis and intracellular calcium homoeostasis our findings support the involvement of SLC10A7 in GAG biosynthesis and specifically in skeletal and tooth development Morphological and skeletal features of SLC10A7-deficient patients a Skull X-ray at 6 years of age (Patient 5) Note the Swedish key appearance of the proximal femur (medial beak and exaggerated trochanters c Hand X-rays at 6 months of age (Patient 2) d Hand X-rays at 3 years of age (Patient 3) the advanced carpal ossification (presence of three ossified carpal centres at 6 months and seven ossified carpal centres at 3 years instead of one and three e Knee at 3 years 9 months of age (Patient 3) Note the genu valgum (angled in knee) and flat epiphyses (arrow) f Spine X-rays at 1 month of age (Patient 1) Note the coronal clefts and irregular shape of vertebrae (arrow) g Spine and hip X-rays at 9 years of age (Patient 4) Informed consent for publication of images was obtained from all individuals or the legal guardians of minors h Localization of the five SLC10A7 mutations relative to the SLC10A7 gene organization (striped rectangles indicate the 5′ and 3′-UTRs) c In situ hybridization analysis of Slc10a7 mRNA expression in E14.5 mouse embryos and P10 mouse tissues The blue staining indicates sites of RNA hybridization empty arrows indicate specific staining in cartilaginous tissues: Meckel cartilage (left panel) in the mandible and phalanges in the digits (central panel) and vertebrae (right panel) Note the positive staining in the lung on the right panel filled arrows indicate specific staining in the hypertrophic zone of the growth plate in the digits (left panel) in the tarsals (central panel) and in the epiphysis of the humerus (right panel) Slc10a7 mRNA expression was localized to the growth plate of several long bones and was more intense in the chondrocytes of the hypertrophic zone at P0 there was stronger expression in the papillary layer of the oral mucous membrane underneath the palate as well as in the ameloblast layer of emerging teeth Slc10a7−/− mice display skeletal dysplasia with skull anomalies b Measurement of body weight and naso-occipital length (body length) and radiographic assessment of Slc10a7+/+ Slc10a7+/− and Slc10a7−/− mice at birth (a) or at 8 weeks (b) demonstrating that Slc10a7−/− mice exhibited a skeletal dysplasia with a more rounded skull c Evolution of body weight demonstrating the growth delay in Slc10a7−/− mice compared with wild-type littermates d Three-dimensional reconstruction of 8-week-old mouse skulls by μCT analysis and skull length and width measurements demonstrating that Slc10a7−/− skulls are less elongated than wild-type skulls nonsignificant; ***p ≤ 0.001; ****p ≤ 0.0001 (two-tailed t-test) n = 35 (Slc10a7+/−) and n = 19 (Slc10a7−/−) at birth; n = 7 (Slc10a7+/+) n = 7 (Slc10a7+/−) and n = 6 (Slc10a7−/−) at 8 weeks SLC10A7 deficiency leads to enamel anomalies in human and in mice a Intra-oral photography of Patient 4 at 9 years of age showing hypomineralized amelogenesis imperfecta (left panel) X-ray panoramic of Patient 5 at 6 years of age showing absence of enamel radiolucency corresponding to amelogenesis imperfecta associated with severe oligodontia (right panel) b Three-dimensional reconstruction of mandibles from μCT analysis of 8-week-old mouse skulls and volume measurement of mandibles lower incisors and lower molars at 8 weeks nonsignificant; ****p ≤ 0.0001 (two-tailed t-test) c Scanning electron microscopy of mandible incisor from Slc10a7+/+ and Slc10a7−/− mice Low magnification (left panels) shows conservation of enamel morphology but decreased thickness in Slc10a7−/− mice The boxed areas in the left panels are shown at higher magnification (middle and right panels) the aprismatic layer was absent and the external prismatic layer was altered giving a rough aspect to the enamel surface (middle panels: arrows indicate hole in the external prismatic layer; a = aprismatic enamel layer High magnification of internal prismatic enamel shows absence of a well-defined prismatic pattern in Slc10a7−/− mice with fused rods and inter-rod structures (right panels; r = rod These images represent three incisors analysed Slc10a7−/− mice exhibit long-bone macro- and microstructure defects a Alizarin red/Alcian blue staining of newborn femurs and measurement of newborn femur length and femur length/width ratio n = 9 (Slc10a7+/−) and n = 10 (Slc10a7−/−) b Three-dimensional reconstruction of μCT analysis of 8-week-old mouse femurs and measurement of 8-week-old femur length and femur length/width ratio Panels (a) and (b) demonstrate that Slc10a7−/− femurs c Three-dimensional μCT of sections of 8-week-old distal femur metaphyses from Slc10a7+/+ and Slc10a7−/− mice Graphs show trabecular and cortical bone volume (BV/TV) and bone mineral density (BMD) d Safranin O staining of the distal femur epiphysis of newborn Slc10a7+/+ and Slc10a7−/− mice Right panels show higher magnification of the growth plate e Masson’s Trichome staining of distal femur epiphysis of newborn Slc10a7+/+ and Slc10a7−/− mice Images are representative of n = 10 and n = 5 per group for Safranin O and Masson’s Trichome staining nonsignificant; *p ≤ 0.05; **p ≤ 0.0.1; ****p ≤ 0.0001 (two-tailed t-test) The columnar organization of proliferative chondrocytes was visible; however proliferative chondrocytes were more tightly packed and the thickness of the proliferative zone was reduced in Slc10a7−/− mice The prehypertrophic/hypertrophic zone was the most affected layer in Slc10a7−/− growth plates the thickness of the hypertrophic zone in Slc10a7−/− mice was limited to two to three cells with anarchic alignment a denser blue staining (corresponding to collagen fibres) was observed in the growth plates of Slc10a7−/− mice compared with wild type these results suggest an alteration in the composition of extracellular matrix possibly due to a reduced proteoglycan/collagen ratio leading to growth plate disorganization and bone growth delay SLC10A7 deficiency leads to defective GAG and enhanced Ca2+ intake b Total sulfated GAGs and heparan sulfates (HS) were quantified according to the DMMB procedure in extracts of SLC10A7-deficient patient fibroblasts and control fibroblasts (n = 3) (a) or in cartilage extracts from 10-day-old Slc10a7−/− or Slc10a7+/+ mice (n = 5) (b) Proportions of HS are expressed as a percentage of total sulfated GAGs (% HS) c Immunohistofluorescence for HS (red) or CS (red) counterstained with DAPI (blue) on distal femurs of newborn Slc10a7+/+ and Slc10a7−/− mice (n = 5 mice) Arrows indicate more intense CS staining at the close proximity of chondrocytes Graphs show red fluorescent signal intensity in the growth plate for each marker nonsignificant; **p ≤ 0.01 (two-tailed t-test) d A representative recording of intracellular free Ca2+ in SLC10A7-deficient patients fibroblasts and control fibroblasts (n = 3) Fibroblasts were loaded with Fluo-4-AM and preincubated in calcium-free buffer for 30 min before addition of 20 μM CaCl2 *p ≤ 0.05; **p ≤ 0.0.1 (two-tailed t-test) These data provide further evidence that SLC10A7 deficiency results in impaired GAG biosynthesis Two-dimensional electrophoresis showed a shift of the far right haptoglobin glycoforms these results suggest an impact on remodelling of glycans resulting in truncated and abnormal glycan structures This study presents evidence that homozygous mutations in SLC10A7 are responsible for skeletal dysplasia and amelogenesis imperfecta. Five SLC10A7 variants were identified in four patients from four unrelated families and two patients from two distantly related families, segregating according to a recessive mode of inheritance (Supplementary Fig. 1b) SLC10A7 mutations were shown to impair SLC10A7 protein synthesis as evidenced by the reduced signal detected by immunocytofluorescence and western blot analyses Inactivation of Slc10a7 in a mouse model resulted in a complex phenotype including abnormal development of skeletal structures and teeth anomalies that recapitulated the human phenotype supporting the conclusion that SLC10A7 mutations are the cause of the clinical phenotype amelogenesis imperfecta can be considered as a new clinical feature indicative of SLC10A7 mutations SLC10A7 mRNA was more specifically expressed in tissues affected in patients cartilage giving rise to long bones and long-bone growth plates (skeletal dysplasia) lungs and developing heart (congenital heart defect in one patient) strengthening the implication of SLC10A7 deficiency in the occurrence of those clinical features SLC10A7 deficiency was also responsible for an increased Ca2+ intracellular intake in skin fibroblasts confirming a role for SLC10A7 in intracellular calcium homoeostasis as previously suggested by studies in yeast homologues This deregulation of Ca2+ homoeostasis due to SLC10A7 deficiency is most likely responsible for defects in GAG synthesis and glycosylation further analyses of growth factor signalling pathways are necessary to fully understand the pathogenesis of the skeletal dysplasia induced by SLC10A7 deficiency our functional work-up of SLC10A7 has identified a new gene responsible for skeletal dysplasia and amelogenesis imperfecta illustrating the complexity of GAG synthesis and the putative role of Ca2+ homoeostasis in this process thus opening new possibilities for the development of therapeutic approaches by correcting the defective Ca2+ homoeostasis in the Golgi DNA was extracted from venous blood using QIAamp DNA blood Maxi kit (QIAGEN) Fibroblast cultures were established from skin biopsies Exome capture was performed at the genomic platform of the IMAGINE Institute (Paris France) with the SureSelect Human All Exon kit (Agilent Technologies) for the three Turkish patients at the University Medical Center Utrecht and University Medical Center Groningen for the two Dutch patients and at the Institute of Human Genetics at the Julius Maximilians University Würzburg for the Iranian patients Agilent SureSelect Human All Exon (V4) libraries were prepared from 3 μg of genomic DNA sheared with ultrasonicator (Covaris) as recommended by the manufacturer Single-nucleotide variants were called with GATK Unified Genotyper whereas indel calls were made with the GATK IndelGenotyper_v2 All variants with a read coverage ≤ × 2 and a Phred-scaled quality of ≤ 20 were filtered out Variants were annotated and filtered using an in-house annotation software system (Polyweb The analyses focused on non-synonymous variants Variant pathogenicity was evaluated using the prediction algorithms SIFT (cutoff ≤ 0.05) cutoff ≥ 0.447) and Mutation Taster (cutoff: qualitative prediction as pathogenic) The variant frequency in control datasets was assessed All variants were confirmed by Sanger sequencing and segregation was verified The exons and exon–intron boundaries of SLC10A7 were amplified with specific primers (Supplementary Table 1) Amplification products were purified by ExoSapIT (Amersham) and directly sequenced with the Big Dye Terminator Cycle Sequencing Ready Reaction kit v1.1 on an automatic sequencer (3500XL and 3130XL; PE Applied Biosystems) Sequence analyses were performed with the analysis software Sequencing 6 (Applied Biosystems) and Gensearch (PhenoSystems SA) Skin primary fibroblasts (control and case 1 and 3) were cultured in RPMI medium supplemented with 10% fetal calf serum Total RNAs from fibroblast monolayers were extracted using the RNeasy Mini kit (Qiagen) according to the manufacturer’s instructions SLC10A7 cDNA was amplified after reverse transcription of RNA using the forward primer 5′-AAGGATCCCCCTAACAAATATGAGGCTGCTGG-3′ (BamHI restriction site underlined) and the reverse primer 5′-AACTCGAGGTATACTGTCGGCCTTGTCAGCTT-3′ (XhoI restriction site underlined) The resulting amplicons were cloned into pcDNA™3.1/Myc-His A + (Invitrogen) to generate proteins with an in-frame Myc-His Tag and then sequenced to verify the correct insertion COS-1 cells and MG63 cells (ATCC) were cultured in Dulbecco’s modified Eagle’s mmedium supplemented with 10% fetal bovine serum (FBS) Transfections were performed on cells in 24-well plates or in 8-chamber labtek slides (ThermoFisher Scientific) using jetPRIME® transfection reagent (Polyplus Transfection) according to the manufacturer’s instructions cells in 24-well plates were collected 48 h after transfection and lysed in denaturation buffer transfer and immunoblotting were performed according to standard protocols using monoclonal anti-myc (9E10; 1/1000; Santa Cruz Biotechnologies) or monoclonal anti-actin (clone C4; 1/5000; Millipore) primary antibodies and goat anti-mouse HRP-conjugated secondary antibody (1/2000; Novex cells in 8-chamber slides were fixed 48 h after transfection with 4% paraformaldehyde (PFA) at room temperature for 30 min The washed cell layer was incubated sequentially in phosphate-buffered saline (PBS) containing 1% bovine serum albumin (BSA) for 30 min mouse monoclonal anti-myc antibody for 1 h and Alexa Fluor 594 goat anti-mouse IgG (1/200; Life Technologies After mounting in Prolong gold antifade mountant with DAPI (Molecular Probes Life Technologies) cells were observed with an Axioplan2 imaging microscope (Zeiss) Probes for SLC10A7 and Slc10a7 corresponded to nucleotides 46-948 of GenBank accession NM_001029998.5 and nucleotides 429-717 of GenBank accession NM_001009981.2 Synthetic cDNAs (Eurofins Genomics) were used to generate antisense and sense cRNA probes using a SP6/T7 DIG RNA labelling kit (Roche) and digoxigenin-11-UTP (Roche) according to the manufacturer's specifications Paraffin sections of paraformaldehyde-fixed human fetuses at 6 and 7 weeks of gestation (Carnegie stages 16 and 19 respectively; obtained with institutional review board approval) murine wild-type embryos at embryonic age E12.5 and tissues from wild-type newborn and 10-day-old mice were deparaffinized These sections were treated with 20 μg/ml Proteinase K for 8 min at 37 °C and postfixed with 4% PFA for 12 min After washing in PBS and 2 × Saline Sodium Citrate Buffer (SSC) they were acetylated in 0.25% acetic anhydride in 0.1 M triethanolamine for 10 min The sections were hybridised to 5 μg/ml DIG-11-UTP-labelled SLC10A7 or Slc10a7 cRNA probes in hybridization buffer (50% formamide 1 mg/ml Yeast tRNA and 1 × Denhardt’s solution) overnight at 70 °C each slide was washed in 2 × SSC containing 50% formamide at 65 °C for 30 min Each slide was treated with 20 μg/ml RNAse A in TNE (10 mM Tris-HCl (pH 7.6) 50 mM NaCl) at 37 °C for 30 min and washed in TNE the slides were washed twice with 2 × SSC and 0.1  × SSC at room temperature for 15 min each [41] After staining with 5-bromo-4-chloro-3'-indoylphosphate p-toluidine salt and nitroblue tetrazolium chloride in the dark (Roche) according to manufacturer’s recommendations slides were scanned using a Nanozoomer 2.0 scanner (Hamamatsu) and visualized using NDPviewer (Hamamatsu) For analysis of GAG content in primary fibroblasts from patients and controls 7 × 106 cells at confluence in 75 cm2 flasks were washed twice in PBS before treatment with trypsin RPMI medium was added and cells were centrifuged at 2000 × g for 5 min and suspended in 1600 µl of extraction buffer (50 mM Tris-HCl pH 7.9 10 μl was removed for protein quantification with BCA Assay (Thermofisher) Proteins were digested by proteinase K treatment (200 µg/mL) at 56 °C overnight followed by 30 min at 90 °C for inactivation of proteinase K 7.5 U of DNAse I (QIAGEN) was added and the samples incubated overnight at 37 °C samples were centrifuged 10 min at 10,000 × g in Nanosep MF 0.2 µm tubes from Pall Corporation (France) To complete the separation of protein from GAGs NaCl was added at a final concentration of 4 M and samples were vortexed for 30 min Trichloroacetic acid (10% final concentration) was added at 4 °C and samples were stored at this temperature for 15 min The samples were then centrifuged at 10,000 × g for 10 min and the pellet discarded Trichloroacetic acid (TCA) was eliminated by chloroform extraction followed by serial dialyses of the aqueous phase against a Tris buffer (50 mM Tris-HCl 50 mM sodium acetate and 2 mM calcium chloride Samples were lyophilized then dissolved in pure water or in glycanase digestion buffer (100 mM sodium acetate cartilages and skeletal muscle from 10-day-old mice were weighed then suspended in 2 ml of extraction buffer and finally their GAGs were extracted as detailed above for cells Human skin fibroblasts were plated at 15 × 103 cells/cm2 in μ-slide 8-well glass bottom (Ibidi) in RPMI media containing 10% FBS the media was replaced with serum-free fresh media the cells were incubated in buffer containing fluo-4 dye (2 µM) for 30 min Cells were washed three times with Ca2 + -free buffer and then kept in Ca2+-free buffer (140 mM NaCl Under fluorescent microscopy (Nikon Instruments Eclipse Ti Inverted Microscopes CaCl2 (20 mM) was then added extracellularly to facilitate Ca2+ influx Peak fluorescent intensities following CaCl2 were captured and average values from at least two sets of cells per patient or control were obtained for analysis Data were analysed as the percentage of fluorescence intensity before Cacl2 for each cell For analysis of glycoproteins from blood spots on Guthrie cards one circular punched spot was first eluted in 100 μl of distilled water Blood eluate was fivefold diluted in distilled water SDS-PAGE was carried out as described by the manufacturer (Life Technologies) using 4–12% NuPAGE Bis-Tris gels and 2 dimensional gel electrophoresis (2-DE) was carried out on 10 μl of blood eluate as described by the manufacturer (Life Technologies) using ZOOM Strip pH 4–7 for the first dimension and 4–12% NuPAGE Bis-Tris gels for the second dimension separated proteins were transferred to nitrocellulose (100 Volts 1 h) and protein glycoforms haptoglobin (Hpt) and orosomucoid (oroso) were identified using the following rabbit primary antibodies: anti-haptoglobin (Dako catalogue number A0030; 1/5000 in Tris-Tween-Buffer-Saline); anti-orosomucoid (Dako catalogue number Q0326; 1/2000 in Tris-Tween-Buffer-Saline (TTBS); anti-transferrin (Siemens catalogue number OSAX15; 1/4000 in TTBS) and anti-α-anti-trypsin (Siemens catalogue number OSA209; 1/10,000 in TTBS) catalogue number NA934V; 1/5000 in TTBS) was used as secondary antibody Images were acquired using a Chemidoc XRS camera system from Bio-Rad All procedures were performed in accordance with the guidelines for animal care of French Animal Care and Use Committee Slc10a7tm1a(EUCOMM)Hmgu ES cells (MGI:1924025) from the EuMMCR were injected into blastocysts from grey C57Bl/6N mice by PolyGene AG The resulting chimerae were bred with C57Bl/6N mice to obtain Slc10a7tm1a(EUCOMM)Hmgu/+ mice (referred to as Slc10a7+/−) The mice were genotyped using the following primers: 5′-CCGCTTCCTCGTGCTTTAGGTA-3′ and 5′-AACCTCTACAGATGTGATATGGCTG-3′ for transgenic allele amplification and 5′-GAATCCAGTACAGGAGAGCCACAT-3′ and 5′-TAGAGACCAGGAATTCTGCTAGACA-3′ for wild-type allele amplification the date of vaginal plug was designated E (embryonic age) 0.5 For all analyses of Slc10a7+/− and Slc10a7−/− mice wild-type littermates were used as controls and both male and female were used at birth cleared by KOH treatment and stored in glycerol according to standard protocols Images were captured with an Olympus SZX12 stereo-microscope Bone sizes were measured using ImageJ software Whole mouse skeletons were radiographed using a Faxitron (MX-20) For μCT analyses adult animals were euthanized and femurs and heads were isolated stripped of soft tissue and stored in 70% ethanol Three-dimensional microarchitecture of the distal femur was evaluated using a high-resolution Skyscan1076 microtomographic imaging system (Skyscan Three-dimensional reconstructions were generated using NRecon software (Skyscan) Trabecular and cortical measurements were obtained using CTan software (Skyscan) on a set of sections located within the secondary spongiosa under the growth plate and under the secondary spongiosa The measured volume was chosen to be proportional to the femur length Avizo software (FEI Visualization Sciences Group MA) was used for three-dimensional visualization of heads and for mandible incisor and molars selection and volume quantification Mandibles from 8-week-old Slc10a7−/− and Slc10a7+/+ mice (three of each genotype) were dissected out and stored in 70% ethanol Incisors were cut along the frontal axis at the level of bone emergence using a rotating diamond wheel and optically controlled monitoring Previous microscanner analysis was used to validate the cutting axis (perpendicular to incisor axis) in order to analyse the buccal part of the incisor Sample surfaces were polished with sandpaper of successively decreasing grits Conditioning of the enamel surface was achieved by etching with 37% phosphoric acid for 30 s and carefully drying Each sample was observed with a scanning electron microscope (TM3030 Incisor morphology was used as a criterion for calibration of section planes Newborn femurs were fixed in 4% paraformaldehyde decalcified with 0.4 M EDTA before paraffin embedding and 5 μm sections were used for Safranin O and Masson’s trichrome staining or immunofluorescence sections were subjected to digestion with 0.5 U/ml chondroitinase ABC (Sigma-Aldrich) for 2 h at 37 °C and catalogue number 370255-1) and anti-CS (1/200 Alexa fluor 594 goat anti-mouse was used as secondary antibody and slides were mounted in Prolong Gold Antifade with DAPI mounting medium (1/200; Life Technologies catalogue number A11005) and scanned using a Nanozoomer 2.0 (Hamamatsu) Specific signal intensity was measured with ImageJ software selecting equivalent areas of the growth plate in each group Statistical analyses were performed using GraphPad PRISM Statistical differences between two groups were analysed with a two-tailed Student’s t-test A p-value of < 0.05 was considered statistically significant The authors declare that all the data supporting the findings of this study are available within the paper and its Supplementary Information files Nosology and classification of genetic skeletal disorders: 2015 revision Chondrodysplasia with multiple dislocations: comprehensive study of a series of 30 cases XYLT1 mutations in Desbuquois dysplasia type 2 Identification of CANT1 mutations in Desbuquois dysplasia Expanding the clinical spectrum of B4GALT7 deficiency: homozygous p.R270C mutation with founder effect causes Larsen of Reunion Island syndrome Mutations in biosynthetic enzymes for the protein linker region of chondroitin/dermatan/heparan sulfate cause skeletal and skin dysplasias Abnormal proteoglycan synthesis due to gene defects causes skeletal diseases with overlapping phenotypes Loss of chondroitin 6-O-sulfotransferase-1 function results in severe human chondrodysplasia with progressive spinal involvement Loss of dermatan-4-sulfotransferase 1 function results in adducted thumb-clubfoot syndrome Loss of dermatan sulfate epimerase (DSE) function results in musculocontractural Ehlers-Danlos syndrome Dysplastic spondylolysis is caused by mutations in the diastrophic dysplasia sulfate transporter gene Determinants of glycosaminoglycan (GAG) structure Further delineation of CANT1 phenotypic spectrum and demonstration of its role in proteoglycan synthesis Molecular and phylogenetic characterization of a novel putative membrane transporter (SLC10A7) The plasma membrane protein Rch1 is a negative regulator of cytosolic calcium homeostasis and positively regulated by the calcium/calcineurin signaling pathway in budding yeast CaRch1p does not functionally interact with the high-affinity Ca(2 + ) influx system (HACS) of Candida albicans Molecular cloning and characterization of a novel human C4orf13 gene tentatively a member of the sodium bile acid cotransporter family High-throughput screening of mouse gene knockouts identifies established and novel skeletal phenotypes A syndrome of joint laxity and impaired tendon integrity in lumican- and fibromodulin-deficient mice in Essentials of Glycobiology 2nd edn (eds Regulation of intra-Golgi membrane transport by calcium on synthesis and secretion of cartilage collagen and proteoglycan Heparan sulphate proteoglycans on rat parathyroid cells recycle in low Ca2 + medium The release of parathyroid hormone and the exocytosis of a proteoglycan are modulated by extracellular Ca2 + in a similar manner Heparan sulphate proteoglycans fine-tune mammalian physiology Functional overlap between chondroitin and heparan sulfate proteoglycans during VEGF-induced sprouting angiogenesis Mutations in EXTL3 cause neuro-immuno-skeletal dysplasia syndrome Two-dimensional electrophoresis highlights haptoglobin beta chain as an additional biomarker of congenital disorders of glycosylation Developmental regulation of the growth plate Ext1-dependent heparan sulfate regulates the range of Ihh signaling during endochondral ossification Sulfation of chondroitin sulfate proteoglycans is necessary for proper Indian hedgehog signaling in the developing growth plate conserved regulator of early chondrocyte maturation and skeletal length Morphogenetic roles of perlecan in the tooth enamel organ: an analysis of overexpression using transgenic mice Targeted disruption of two small leucine-rich proteoglycans excerpts divergent effects on enamel and dentin formation Hypomaturation amelogenesis imperfecta caused by a novel SLC24A4 mutation New tools for Mendelian disease gene identification: PhenoDB variant analysis module; and GeneMatcher a web-based tool for linking investigators with an interest in the same gene Fast and accurate long-read alignment with Burrows-Wheeler transform The Genome Analysis Toolkit: a MapReduce framework for analyzing next-generation DNA sequencing data The Sequence Alignment/Map format and SAMtools ADAMTSL2 mutations in geleophysic dysplasia demonstrate a role for ADAMTS-like proteins in TGF-beta bioavailability regulation Improved and simple micro assay for sulfated glycosaminoglycans quantification in biological extracts and its use in skin and muscle tissue studies Age-related changes in rat myocardium involve altered capacities of glycosaminoglycans to potentiate growth factor functions and heparan sulfate-altered sulfation Glycosaminoglycan blotting and detection after electrophoresis separation Download references This work was supported by the European Community’s Seventh Framework Programme (FP7/2007-2013) under grant agreement number 602300 (SYBIL) and the Fondation pour la Recherche Médicale (FRM) funding (DEQ20120323703) the Genomic and Bioinformatic facilities of Institut Imagine for help in exome sequencing analyses Morad Bensidhoum and SFR IMOSAR for μCT scans Benjamin Fournier from Laboratory of Molecular Oral Pathophysiology (INSERM UMR_S1138) for scanning electron microscopy analysis Barbara Vona and Thomas Haaf from Institute of Human Genetics of Julius Maximilians University Würzburg (Germany) Eric Hennekam from the department of genetics (University Medical Center Utrecht) and the medical specialists in the UMC Utrecht Expert Center for Congenital Orofacial and Dental Anomalies for sharing patient data and the Histology facility and the Imaging facility of SFR Necker Foulcer for providing helpful comments on the manuscript Université Paris Descartes-Sorbonne Paris Cité Muriel De La Dure-Molla & Valérie Cormier-Daire Cell Growth and Tissue Repair CRRET Laboratory Sandrine Chantepie & Dulce Papy-Garcia Akdeniz University Paediatric Genetic Deaprtment Laboratory of Embryology and Genetics of Congenital Malformations Reza Maroofian & Ehsan Ghayoor Karimiani Laboratory of Molecular Oral Pathophysiology designed and performed all functional studies and mouse phenotype analyses performed exome sequencing analysis and molecular studies contributed to the generation of the mouse model contributed to the maxilla and dental phenotyping in human and mouse wrote the manuscript and all the co-authors reviewed the manucript The authors declare no competing interests Publisher's note: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations Download citation DOI: https://doi.org/10.1038/s41467-018-05191-8 Anyone you share the following link with will be able to read this content: Sorry, a shareable link is not currently available for this article. 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Metrics details An Author Correction to this article was published on 02 March 2020 This article has been updated The use of functional information in the form of species traits plays an important role in explaining biodiversity patterns and responses to environmental changes Although relationships between species composition and the environment have been extensively studied on a case-by-case basis and it remains unclear how generalizable these relationships are across ecosystems we collated 80 datasets from trait-based studies into a global database for metaCommunity Ecology: Species Each dataset includes four matrices: species community abundances or presences/absences across multiple sites environmental variables and spatial coordinates of the sampling sites The CESTES database is a live database: it will be maintained and expanded in the future as new datasets become available the CESTES database provides an important opportunity for synthetic trait-based research in community ecology species abundance • species trait • environmental feature • latitude • longitude year of data collection • type of ecosystem • level of human disturbance terrestrial natural environment • fresh water • marine biome • anthropogenic terrestrial biome • natural environment • area of mixed forest • cultivated environment Machine-accessible metadata file describing the reported data: https://doi.org/10.6084/m9.figshare.11317790 we know much less about other groups and ecosystem types Upper panel: Map of the 80 dataset locations over the globe (blue spots) (the orange smaller spots represent the 10 ancillary datasets from ceste the non-spatial supplement of CESTES - see the Methods section); the four coloured polygons represent four datasets that are covering continental extents The background world map is from OpenStreetMap contributors Bottom panel: Bar plots and histogram describing the content of the database in terms of: study group The CESTES database aims to significantly contribute to research in biogeography macroecology (including in complement with phylogenies) the quality of its content and structure will allow meta-analyses and syntheses (e.g. the role of taxonomic and functional diversity in spatial patterns of communities) specific datasets will enable the exploration of new questions on a given group The rationale for developing the CESTES database is generally for the study of TER in relation to metacommunity ecology and/or macroecological questions we focussed on datasets that were appropriate within the metacommunity or macroecology context (i.e species assemblages distributed across space) and that focussed on traits to understand biodiversity patterns and responses This prerequisite led us to identify multivariate trait-based studies as the most relevant and rich source of datasets that could fulfil these two requirements Given the complexity that still pertains to trait typology13 we did not restrict ourselves to any specific definition of traits and integrated all possible species characteristics if they were used as “traits” in the original study CESTES users can select traits according to their study needs We identified eligible datasets based on two strategies: 1 aiming to initiate the database construction along a structured workflow aiming to extend the database and open the sharing possibilities if the datasets fulfilled the CESTES requirements The main condition for dataset eligibility was that the TER was the focus of the study and data use the trait and the taxonomic information were collected from similar biogeographic areas (minimizing mismatches between the geographic origins of trait and taxonomic data) the sampled sites were associated with contextual environmental information that was relevant to the community and traits under study The “RLQ” refers to a co-inertia analysis that summarizes the overall link between the three matrices of species abundances/presences-absences (L) The “fourth-corner” refers to a permutation analysis of these three matrices that tests individual trait-environment relationships The use of RLQ and fourth-corner analyses on the datasets ensures that all of them: 1 are multivariate and include both several species and several sites (potentially including spatial information) to align with a metacommunity-like structure have a comparable structure and can be used in comparative analyses and syntheses ALL = (“fourth-corner” OR “fourth corner” OR “fourthcorner” OR “RLQ”) although this literature search strategy was well suited for identifying sources of multivariate datasets In order to relax the constraints due to this specificity we complemented the data search by a networking strategy (see Networking section) could sometimes be reconstructed from the maps presented in the publications medical and simulation studies were not considered we identified a subset of 105 eligible datasets The network strategy took place in parallel to the data search and relied on both formal and informal communications and exchanges with colleagues through conferences This allowed us to identify new data providers or new datasets that we had not found via the earlier literature search we identified an additional set of 34 potentially eligible datasets 7.2% of the datasets were available on the online supplementary materials of the publication These were downloaded and formatted for CESTES’ purposes Success rates of the data search and request Barplot showing the percentage of the different outputs from the data collection process Percentages are calculated from a total of 139 datasets identified as eligible for the CESTES database (based on literature search and networking) Incomplete data mainly refer to the datasets that had no spatial coordinates (ceste) or provided insufficient metadata information (“Agreed but did not share” refers to authors who replied positively to the first request but then never sent their data despite reminders because e.g. they did not find time to prepare the data) Because we received 10 valuable datasets that had no spatial coordinates we decided to open the ceste subsection of the CESTES database and populate it with these specific datasets Some of them could be upgraded to CESTES database when the authors are able to provide the coordinates We downloaded and received datasets in various formats (.doc, .pdf, .csv, .RData, .txt, .shp, etc.). Following Broman & Woo27 we harmonized and gathered them in Excel files This was the most convenient storage format for creating multiple sheets (community handling heterogeneous types of information and building metadata specific to each dataset This storage solution also facilitated visual checking and cleaning of the data records CESTES provides both the processed and the unprocessed (i.e species that are recorded in none of the sites of the study area) and no NAs (Not Available information) in the matrices NA removal was based on a compromise in the relative frequency of NAs in the rows and columns of each table; when too many sites compared to the sample size (e.g >50% of the sites) had NAs for one single variable <30% of the sites) showing NAs for more than one variable we removed those sites instead of removing the variables Since CESTES is primarily designed for trait-based analyses we removed a trait when it included too many NAs across species (i.e when the trait value was NA for more than 50% of the species in the community) or too incomplete trait information was available (i.e when keeping the species would have implied to lose several traits) This was the case for 29 datasets out of the 80 The number of species removed varied from 1 to 209 species (mean = 27 sd = 45) that represented from 1 to 72% of the initial species pool (mean = sd = 17%) (Note that this high maximum value is due to only one single dataset where trait data were exceptionally limiting and implied to remove an important number of species without trait information) When this overall cleaning procedure implied removing any of the species we kept the information of the original unprocessed tables within the Excel file in separate sheets the user can either directly use the processed sheets (“comm” or the original ones and apply any other filtering strategies we make sure that CESTES is flexible depending on the users’ goals and needs Cleaning steps that altered the original dataset (other than formatting) are reported in the “Notes” sheet so that the user can trace back what has been done over the data processing When the data included several temporal horizons (sampling years, or seasons treated as different replicates in the original publication), we split them into different datasets for each time horizon to facilitate further analyses. This explains why several datasets can correspond to one single study area (see Online-only Table 1 attached to this manuscript “DataKey” structure and example of metadata information in CESTES datasets A description is given when the variable full name is not self-explanatory or when potentially relevant information was available Possible empty cells are due to lack of information that could not be recovered from the original publication nor from the data owners We stored the CESTES database via three different storage systems and two types of formats to provide the users with several alternatives in accessing and using the data and R scripts following the updates when new datasets are integrated A zipped folder called “CESTES.zip” includes two alternative formats for the CESTES database: a “xCESTES” folder that includes 80 Excel files (one file per dataset) each named according to the following structure: “AuthorPublicationYear.xlsx” a “rCESTES” folder that includes the CESTES core processed database (comm coord matrices) as an R list object “CESTES.RData” plus two R scripts and two metadata tables for data processing and exploration (see Usage Notes section) an extended metadata table, “CESTES_metadata.xlsx”, that provides the general metadata information of all the datasets (i.e., combining the information from the Online-only Tables 12 of this Data Descriptor) a tutorial document, “HOW_TO_SHARE_MY_DATA_FOR_CESTES.pdf”, that explains how to share data for integrating future datasets in the database (see Supplementary File 1) unprocessed files as they were provided by the data owners (thus possibly in different formats are available by request to the corresponding author The 80 files currently in CESTES are structured into at least 8 sheets, depending on the original information and specificities of each dataset (Fig. 1) The first four sheets include the processed core-data themselves: “comm”: matrix of species abundances (68) or presences/absences (12) with species in columns and sites in rows (species are sometimes OTUs in some groups such as phytoplankton or genus in some groups such as macroinvertebrates “traits”: matrix of species trait information with traits in columns and species in rows “envir”: matrix of environmental variables in the broad sense of environment any type of biotic and abiotic conditions or habitat characteristics relevant to the community of interest according to the original publication with variables in columns and sites in rows the latitude as columns (in the Geographical Coordinate System as used in the original study) and sites in rows In every dataset, a “DataKey” sheet provides a description of all the entries of the four matrices (Fig. 4) Specific comments and information about any alteration applied to the dataset can be found in the “Notes” sheet or variables that were removed due to missing information how the trait values were averaged across species when several measurements were available how the original dataset was split into several datasets when there were several sampling periods The contact person for each dataset is also specified at the top of the “Notes” sheet of the dataset When the cleaning procedure implied changing the original datasets (see Data processing section above) we kept the information of the unaltered tables within the Excel file in separate sheets: “commfull” The “splist” sheet includes the full list of taxa and the “sitelist” sheet Both can provide additional information about the species (e.g taxonomic classification) and the sites (e.g regional information) when specified by the authors Note that the species (site) names might not appear in the “splist” (“sitelist”) of all the datasets; this is because some authors preferred to provide their data in a redacted form by censoring the species or the site names As this does not hamper most of the analyses in community ecology these datasets were integrated in the database The CESTES database includes 80 datasets that cover different areas of the globe, ecosystem types, taxonomic groups, and spatial extents (Fig. 1). An overview of these datasets is presented in the Online-only Table 1 We provide access to 10 additional datasets that were not completely suitable for the CESTES database, due to the absence of spatial information or insufficient metadata but that were potentially valuable for their three other data matrices (see Online-only Table 3 attached to this manuscript) except that they do not present the “coord” sheet and sometimes include only partial metadata Some of the ceste datasets are likely to be enhanced in the near future and upgraded to the CESTES database as soon as they are made complete ceste is stored in a zipped folder named “ceste.zip” that includes a series of 11 Excel files (10 data files + 1 metadata file) and can be found at the following links: Figshare: https://doi.org/10.6084/m9.figshare.c.4459637 -iDiv Biodiversity Portal: https://doi.org/10.25829/idiv.286-21-2695 (under the Attachments tab) The current CESTES database is the starting point of a broader data-sharing project that aims to continue integrating new data as they become available and as new contributors join the consortium by sharing their data In order to maintain the CESTES database in the future we set up three measures to facilitate the data exchange and communication about the database: a project website that advertises the database project and fosters data sharing: https://icestes.github.io/ a tutorial to guide people on how to share their data (Supp. Mat. 1; https://icestes.github.io/sharedata) a designated email address where people can send their data and ask questions about the CESTES project (cestes@idiv.de) and integrated in the database through the iDiv Biodiversity Portal This will update the database and generate a new DOI for the whole updated database ensuring the new contributors are acknowledged The technical validity of the CESTES database relies on five qualities pertaining to the datasets and the overall database: the datasets (1) have individually been subject to peer-review process (3) have been thoroughly checked and cleaned are ready-to-use for analyses and accompanied with metadata information; and the database (4) has a wide taxonomic and geographical coverage and (5) will keep on extending in the future All the datasets included in CESTES had already been the subject of publication(s) in peer reviewed scientific journals, or PhD theses (see Online-only Table 2) each of the dataset has already received technical validation through both analysis and evaluation since the focus of those studies was the species trait-environment relationships the choice of the traits and environmental variables has already been the result of scientific reflection by the authors about the potential relevance of these variables with respect to the ecological context and the scale of study Data content of the CESTES database. Distribution of the number of environmental, site, species and trait variables across the datasets. The curves represent their observed thresholds of Type II error rates - red = 30% The datasets that fall below these thresholds are theoretically exposed to respectively 30% 5% or 0% chance to fail to detect significant TERs with fourth-corner analysis although these exist The figure shows that the majority of the CESTES datasets fall in a medium (70%) to very good (>95%) power zone (Power = 100% − Type II error) This would allow extending CESTES’ potential for synthesis work aiming to bridge metacommunity ecology and biodiversity-ecosystem functioning research In complement to the Excel version of CESTES, the database has also been stored as an.RData object to facilitate its further use for analyses in R109 Each element of the first order list refers to one dataset which itself is a list of four matrices; $comm We set up R code routines (“CESTES_DataPrep.R”) that perform a thorough checking of the matrices especially the match between the matrices’ dimensions and coordinates variables were of mixed types (binary To make the datasets properly readable and analysable by R we made sure the numerical variables were treated as such by the program We also re-coded the binary variables into 0/1 (numeric) the character and nominal variables into factors (this option can be turned off in the function) when made explicit in the original publication) the numeric integer variables into ordinal variables (ordered factors) Our R code routines generate data reports and send them to the working directory in the form of .txt files These give the user different information on the “comm” “envir” and “coord” components of each dataset: list of variables and their types (factor minimum and maximum value of the community data (that allows checking e.g. whether data are abundances or presences/absences) The R code also applies some data transformation (e.g Moran Eigenvector Maps) and calculates some usual trait diversity metrics (e.g All the R functions coded and used for the data preparation are provided in an R script “CESTES_DataPrep.R” A fully processed and “ready-to-use” version of the CESTES database is stored as an .RData object called “CESTES.RData” further data plotting and metadata exploration are made possible via the R script “CESTES_Plots.R” and the two metadata .csv files (“ListDat.csv” and the two metadata files) are stored in a zipped folder called “rCESTES.zip” in the “CESTES” folder at the following links: Figshare (fixed version): https://doi.org/10.6084/m9.figshare.c.4459637 iDiv Biodiversity portal (evolutive version): https://doi.org/10.25829/idiv.286-21-2695 (under the Primary Data tab) The flexibility of the iDiv Biodiversity Portal storage will allow us to keep updating extending and sustaining the CESTES database and the R scripts in the future It comes with R code scripts that allow further checking transforming and exploring the database content (for more details We provide all this information in a folder called “rCESTES.zip” within the “CESTES” folder at the following links: Figshare repository (fixed version): https://doi.org/10.6084/m9.figshare.c.4459637 iDiv Biodiversity Portal (evolutive version): https://doi.org/10.25829/idiv.286-21-2695 A Correction to this paper has been published: https://doi.org/10.1038/s41597-020-0420-z The Theory of Ecological Communities (MPB-57) Beyond species: functional diversity and the maintenance of ecological processes and services Predicting changes in community composition and ecosystem functioning from plant traits: revisiting the Holy Grail Rebuilding community ecology from functional traits Predicting communities from functional traits Revisiting the Holy Grail: using plant functional traits to understand ecological processes Spatial mismatch and congruence between taxonomic phylogenetic and functional diversity: the need for integrative conservation strategies in a changing world Beyond taxonomic diversity patterns: how do α β and γ components of bird functional and phylogenetic diversity respond to environmental gradients across France Low Functional β-Diversity Despite High Taxonomic β-Diversity among Tropical Estuarine Fish Communities Functional and phylogenetic diversity as predictors of biodiversity—ecosystem-function relationships A functional approach reveals community responses to disturbances The global spectrum of plant form and function Matching species traits to environmental variables: a new three-table ordination method Schmidt-Kloiber, A. & Hering, D. 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Bird species and traits associated with logged and unlogged forest in Borneo. figshare, https://doi.org/10.6084/m9.figshare.c.3293726.v1 (2016) Changes in the size structure of carabid communities in forest ecosystems under technogenic transformation Variation in the diversity and composition of benthic taxa as a function of distance offshore depth and exposure in the Spermonde Archipelago Using life-history traits to explain bird population responses to changing weather variability Relating species traits to environmental variables in Indonesian coral reef sponge assemblages Post-fire succession of collembolan communities in a northern hardwood forest A unimodal species response model relating traits to environment with application to phytoplankton communities Trait-Environment Relationships and Tiered Forward Model Selection in Linear Mixed Models Boreal peat properties link to plant functional traits of ecosystem engineers gaps and uncertainties in global plant occurrence information A plant growth form dataset for the New World How do traits vary across ecological scales The return of the variance: intraspecific variability in community ecology Elevation matters: contrasting effects of climate change on the vegetation development at different elevations in the Bavarian Alps Integrating trait and phylogenetic distances to assess scale-dependent community assembly processes R: A Language and Environment for Statistical Computing Download references Jeliazkov were collected with financial support from the Fédération d’Ile-de-France pour la Recherche en Environnement (FIRE FR-3020) Tejerina-Garro had financial support from the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq No Menz were collected with financial support from the Swiss National Science Foundation (grants 31003A_125398/2 and 31003A_149656) awarded to RA Present address: Gerencia de Planificación y Gestión Hídrica German Centre for Integrative Biodiversity Research (iDiv) Department of Evolutionary Biology and Environmental Studies Centre d’Ecologie et des Sciences de la Conservation (CESCO) University of South Bohemia in Ceske Budejovice Institut de Recerca de la Biodiversitat (IRBio) Unité Écologie et Modèles pour l’Halieutique Programa de Pós-Graduação em Recursos Naturais do Cerrado (RENAC) Campus de Ciências Exatas e Tecnológicas - Henrique Santillo CFE- Centre for Functional Ecology - Science for People & the Planet Institute of Environmental Protection and Engineering ARC Centre of Excellence for Coral Reef Studies Tropical Island Sustainable Development Research Center National Penghu University of Science and Technology Programa de Pós-Graduação em Ecologia de Ambientes Aquáticos Continentais Department of Environmental and Forest Biology College of Environmental Science and Forestry University of Applied Sciences HTW Dresden Biological Dynamics of Forest Fragments Project National Institute for Amazonian Research and Smithsonian Tropical Research Institute Domingo Flores Hernandez & Julia Ramos Miranda Institut de recherche en biologie végétale Environment and Evolution and Centre for Future Landscapes Ecological Synthesis and Biodiversity Conservation Lab Swiss Institute for Dryland Environmental and Energy Research Jacob Blaustein Institutes for Desert Research Royal Belgian Institute of Natural Sciences Department of Migration and Immuno-ecology Laboratoire Évolution et Diversité Biologique Swiss Federal Institute of Water Science and Technology Department of Physical Geography and Ecosystem Science CNRS - Université de Montpellier - Université Paul-Valéry Montpellier - EPHE Institute of Evolutionary Biology (CSIC-Universitat Pompeu Fabra) University of Colorado Department of Ecology and Evolutionary Biology School of Geography and Environmental Science Institute of Parasitology and Institute of Zoology Programa de Pós-Graduação em Sociedade Tecnologia e Meio Ambiente Fenner School of Environment & Society Jonathan Chase (J.C.) and Alienor Jeliazkov (A.J.) conceived the idea of the database and Darko Mijatovic (D.M.) prepared the metadata coded all the R code routines and drafted the manuscript and Stéphane Chantepie (S.C.) built the CESTES website All authors contributed with data and revisions to the manuscript Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations The Creative Commons Public Domain Dedication waiver http://creativecommons.org/publicdomain/zero/1.0/ applies to the metadata files associated with this article Download citation DOI: https://doi.org/10.1038/s41597-019-0344-7 a shareable link is not currently available for this article Sign up for the Nature Briefing newsletter — what matters in science I thought as we inched along a narrow London street in our newly acquired campervan and when he parked it outside our house the front room went dark It was as long as a canal boat and as wide and high as a fire engine and as we moved gingerly between the ranks of parked cars early on that midsummer morning The van was to be our house and sole means of transport as the five of us – two adults six and three – travelled around France over the following fortnight had not stinted in creating a "home": into the E495's broad acres they had fitted a living room as well as a cooker with two ovens and a microwave a sink with hot and cold water and a domestic-size fridge-freezer we initially found the E495 less attractive there was a blind spot behind that would hide a small Benelux country Reversing could only be achieved with the help of someone outside shouting and waving We had just escaped the narrow streets of Hackney and joined the roaring traffic on the northern approach to the Blackwall Tunnel under the Thames when my partner remembered something Bernie had said about the E495 being too big to get through As we rushed southwards beneath gantries that seemed almost to scrape the bouffant sweep of our roof she dug through the owner's manual to find out exactly how high the vehicle was Nearing the dark "O" of the tunnel's mouth bellowing "I'm going through behind him!" and prepared for the crunch of twisting metal "I think Bernie said you won't fit in the outside lane northbound." We settled into a steady cruising speed across the golden fields of Kent and began to calm down Once I grew used to doing everything slowly and checking each mirror before moving we found our first campsite in a wooded valley on the outskirts of Honfleur and were allocated a patch of lawn about eight metres by eight "Explore!" The eldest loitered suspiciously around the van steps while the middle one kicked a football around until it went deep beneath the Belgian caravan next door There was a certain amount of business to do to set up camp find the electricity point and plug in the extension Then we unpacked a groundsheet and a folding picnic table and scattered them about the lawn to demonstrate ownership I think how green we must have seemed that first night As we walked around campsites over the coming days I learned how the established camper makes themselves feel at home the motorhome owner will typically spread a patio-sized groundsheet outside their front door and pull a loggia from the side of their van before putting up a tent or two for extra storage followed by a large family dining table with an oilcloth and wine glasses and cutlery and lanterns The TV satellite dish is then hoisted and aligned and windbreaks and hammocks pegged down and strung out Then the outdoor lifestyle gear can be brought down from the van Only when the grass is almost invisible beneath the mass of equipment can a plot truly be called home we bought bodyboards and wetsuits and beachballs and an inflatable boat and when we arrived in a new corner of France I would find myself roping it off with washing line and hanging bone dry towels on it to block the views of passersby and show – what we hired bicycles and rode along newly laid-out cycle routes over bridges and pontoons raised above the canals and marshland stopping for lunch in a bistro in the square of a small town kitesurfed and walked through a pine forest with steps down to a private beach where a pelting surf provided enough entertainment for most of a day In the late afternoon at each place we would return to the campsite to swim in the pool then sit and watch the sun turn the sea the colour of molten steel I shall now tell you briefly about the toilet incident The loo worked like this: the bowl emptied into a cassette which had a "blade" that closed to seal the smells off When the cassette was full a light glowed on the cistern Bernie had said to remove it and take it to the disposal point and equipped with wheels and an extendable handle so it looked like a piece of walk-on luggage the men could be seen walking around with the family sewage and after a week of abuse by three children ours went wrong The only way to fix it was to take the cassette out with the blade open which led to a sort of avalanche that filled the little well on the side of the vehicle I spent much of that afternoon walking between toilet block and van with a bucket and black smiling at my fellow campers and at the tradespeople who were setting up an antiques market next to our pitch I imposed a ban on using the loo after that By now we felt ourselves to be real campers Out on the road we would diligently return the open-handed campervan wave And because we were driving the Bessacarr E495 – the Cadillac of European motorhoming some might say – aficionados were keen to chat Once we went into a service station and came out to find a couple examining its every angle in an admiring way we had a look at one of these," said the man "My wife's worried she might be a bit too big Through these conversations we glimpsed a whole holiday culture that had been obscure to us hidden within the great white sheds that are to be found on the motorways of Europe through the summer months The people inside these vehicles are mostly families or retired people who are getting away economically and not very quickly Some travel year after year to the same spot where they meet up with friends they have made the previous year In Biarritz we met a Welsh family who had returned to the same site every summer for eight years and the company towed it to the site every year before they arrived and took it away when they went home again Their children would text their summer friends to ask when they were getting to Biarritz and how long they were going to be there A retired German couple told us that until their family had grown up they had spent all of their summer holidays caravanning most economical way for a family to see Europe," said the man We met the Germans in a campsite in a pine forest west of Bordeaux Here were all the usual facilities – a pool swings – but also a beach that runs for a hundred miles along the Atlantic coast reached from the site by tumbling down the great dune We slung our hammock between the trees and strung out our washing lines and went exploring we ate in the restaurant with views out to the Cap Ferret watching paragliders play in the air currents that rose up from the beach and pleasure boats sailing home to Arcachon and we swam almost alone off the beach before climbing the hill back to the campervan We came back from our holiday browner and fitter and better fed having seen parts of France we would not otherwise have thought to visit We were sad to give our motorhome back to Bernie We loved the sense of freedom it had given us So much that we are now thinking of buying one – one that's much older Holiday On Wheels (+44 (0)1440 761 725, holidayonwheels.co.uk) rents a variety of fully-equipped campervans, from £650 per week. Go Motorhome Hire (+44 (0)845 686 4473, go-motorhomehire.co.uk) rents vans from £700 per week On touring: discovertouring.co.uk. The International Caravan & Motorhome 2009 show (+44 (0)871 230 5575, caravanshows.com) takes place at the NEC The aesthetic and artistic environment of her upbringing and her classical French heritage gave her a natural love of beauty and influenced her work she wanted to achieve with these handbags what a modern and elegant woman would want Carmin handbags are created for women who love style She believes the future of luxury is about aesthetic being the reflection of herself and hiding her most valuable treasures and secrets The opinions expressed in this article are ours Luxurylaunches.com is an award winning premium lifestyle website It features the latest and the best from the world of extravagance and opulence