CLICK HERE TO SUBSCRIBE TO THE TDN FOR FREE! Remember Ben with a gift to Thoroughbred aftercare Subscribe for FREE to the Daily PDF or the News Alerts Home » Archive » Top News Europe » Group 2 Winner Klaynn And Listed-Placed Thiene Added To Arqana's Vente d'Elevage Group winner Klaynn (Ire) (Make Believe {GB}) and the stakes-placed Thiene (Ire) (Cotai Glory {GB}) are the newest pair of wildcards to be added to the upcoming Arqana Vente d'Elevage and has won the Listed Premio Repubbliche Marinare followed by the G2 Premio Dormello she is a out of a half-sister to three black-type winners including G1 Derby Italiano winner De Sica (Ire) (Sri Pekan) second in the Listed Prix Ceres last weekend will be offered by Alessandro and Giuseppe Botti as lot 140 and is a four-time winner She is out of a half-sister to top-tier winner Vedelago (Ire) (Red Clubs {Ire}) Arqana's Vente d'Elevage will take place in Deauville from Dec Both fillies will sell during the first session on Saturday Not a subscriber? Click here to sign up for the daily PDF or alerts. Copy Article Link Editor / News Stories:editor@thetdn.com Advertising:advertising@thetdn.com Customer Service:customerservice@thetdn.com Click Here to sign up for a free subscription By Courtney Mares for CNA a saint experienced a mystical vision in which the Blessed Virgin Mary placed the Christ Child in his arms as he offered his first Mass in Rome’s Basilica of St The little-known but profound experience of the newborn Jesus occurred within the storied walls of the Marian basilica’s Chapel of the Nativity, where St. Cajetan of Thiene prayed before the relics of Christ’s manger I found myself in the true and material most holy Nativity,” St an Augustinian nun and spiritual confidant whose relics are said to rest in the basilica appeared in the vision and encouraged him to embrace the child “To encourage me was the most blessed Jerome whose remains rest at the entrance of the same crib,” he wrote 6 during the feast days of the Circumcision and Epiphany has remained a central spiritual moment for Congregation of Clerics Regular and deliberately waited for three months in order to celebrate his first Mass on Christmas Eve in the Chapel of the Nativity The choice was deeply symbolic. The Basilica of St. Mary Major has long been a cornerstone of devotion to the Nativity of Christ. Known as “the Bethlehem of the West,” it houses a relic believed to be fragments of the manger in which Jesus was laid which can now be venerated in the crypt beneath the basilica’s main altar Orqueida underlined that devotion to the Nativity of Christ remains a central part of the spirituality of the Theatine order today Cajetan was one of the great reformers of the Church during the period of the Reformation He is remembered as the “saint of divine providence,” a title reflecting his unwavering trust in God He co-founded the Theatine order in 1524 to counter the corruption of his time combining monastic poverty with active ministry caring for the poor and marginalized Cajetan’s humility and austerity: “He was blameless With his own hands he fed them and served them There was a poor straw sack where he rested … His dress was of coarse cloth.” is often depicted in art holding the infant Jesus who also had a mystical experience involving the Christ Child Near Rome’s Piazza Navona, the Basilica of Sant’Andrea della Valle the Theatine order’s basilica in the Eternal City a grand altarpiece painting and a statue depict St the Sant’Andrea basilica hosts an elaborate display of dozens of Nativity scenes Hidden in the crypt of the Chapel of the Nativity Cajetan holding the infant Jesus identifies the sacred spot where the vision occurred Ignatius of Loyola also chose to offer his first Mass in the same chapel in 1538.) As pilgrims gaze upon the marble statue of Cajetan holding the Christ Child they are invited to enter the mystery of the Nativity and embrace the tender and eternal Word made flesh The Theatine Order has offered this prayer to St Cajetan to be prayed in front of any image of the baby Jesus during the Christmas season: wanted to come from the embrace of your mother to those of your priest St who was pleasing to you because of the holiness of his life and the great faith he always had in your providence which is part of the delight of the blessed in heaven and listen to the prayer we send to you from the bottom of our hearts We present to you the filial abandon which St everything we ask in front of your venerated image will be granted to us If you value the news and views Catholic World Report provides, please consider donating to support our efforts Your contribution will help us continue to make CWR available to all readers worldwide for free Click here for more information on donating to CWR. Click here to sign up for our newsletter Becoming the mother of God: Did Mary have a choice 2018 / 03:40 pm (CNA/EWTN News).- Prominent American professor Mary Ann Glendon has resigned from the Board of Superintendence which oversees the Institute of Religious Works “Professor Glendon … […] 2021 / 07:04 am (CNA).- A Vatican official has encouraged Catholics to apply the Church’s social teaching to evaluate proposals for a “Green New Deal.” Archbishop Paul Gallagher, the Vatican Secr… […] Janada Marcus with Pope Francis at the general audience on March 8 All comments posted at Catholic World Report are moderated While vigorous debate is welcome and encouraged please note that in the interest of maintaining a civilized and helpful level of discussion comments containing obscene language or personal attacks—or those that are deemed by the editors to be needlessly combative or inflammatory—will not be published Δdocument.getElementById( "ak_js_1" ).setAttribute( "value" You can see how this popup was set up in our step-by-step guide: https://wppopupmaker.com/guides/auto-opening-announcement-popups/ Sign up to receive a weekly email with news (function($) {window.fnames = new Array(); window.ftypes = new Array();fnames[0]='EMAIL';ftypes[0]='email';fnames[3]='MMERGE3';ftypes[3]='text';fnames[1]='FNAME';ftypes[1]='text';fnames[2]='LNAME';ftypes[2]='text';fnames[4]='MMERGE4';ftypes[4]='text';fnames[5]='MMERGE5';ftypes[5]='text';fnames[6]='MMERGE6';ftypes[6]='number';fnames[7]='MMERGE7';ftypes[7]='radio';}(jQuery));var $mcj = jQuery.noConflict(true); Vicentini of the Thiene protagonists, with the successes in the Under 11 (5-4 in the final against Sarzana) and in the Under 15s (4-2 in the final against Pumas). Sarzana won the Under 13 (1-0 against the Spanish team Saint Ramon), but a title also speaks Versilia with Pumas who won in the Under 17 (3-2 overAll Stars Viareggio Hockey). A true organizational triumph for the Viareggio SPV, led by for 35 years Claudio Bicicchi, who expects a lot from the upcoming works at the "Gymnasium". "The contract for the works has been awarded – he says – renovation, which will include the rink, boiler room, roof, balustrades and locker rooms. We hope they will begin before the summer and be completed before the start of the hockey season." CONTEMPORARY ART MAGAZINE SINCE 1980 More... “Stavo cercando il tuo cuore lì dentro ma ho trovato solo latte” a solo exhibition by Cecilia De Nisco curated by Marta Papini are on display in the spaces of the former Church of the Dimesse complex in Thiene until March 1st Two exhibitions conceived independently but experienced seamlessly: an open invitation to freely explore contemporary art What characterizes the exhibition project of the Bonollo Foundation is the juxtaposition of two independently conceived shows into a continuous viewing experience This approach stems from both practical and identity-driven necessities: on the one hand it pursues the intentions of a couple of collectors who have been dedicated to contemporary art for over thirty years; on the other hand it considers the particularity of the exhibition spaces the cultural scene of Thiene has been enlivened by a diverse internationally oriented program that offers public access to artworks from the Bonollo alongside the promotion of emerging artistic talents curated by Marta Papini courtesy the Artist e Fondazione Sandra e Giancarlo Bonollo The exhibition dedicated to Cecilia De Nisco features seven oil paintings on canvas specifically designed for this exhibition space The artist maintains a consistent formal style based on a blurred contrast between a vibrant blue background and flickering figures Ambiguity and vagueness are recurring characteristics in De Nisco’s work they manifest both in the spatial-temporal settings and in the meaning of the artworks While the soft brushstrokes and dynamic compositions suggest a childlike world of curiosity and dancing around a bonfire and grinning expressions shift everything onto an uncertain terrain imbued with unease exemplified by the large canvas that gives the exhibition its title: “Stavo cercando il tuo cuore lì dentro ma ho trovato solo latte” (2024) The power of De Nisco’s works lies in their ability to project the sense of discomfort expressed by the characters This is achieved by subtly questioning the interpretative automatisms ingrained in Western culture a goal declared at the very beginning of the visit to the Dimesse complex with the work “Marameo” (2024) The arched canvas fits perfectly into the space that once hosted the church’s altarpiece presenting a composition reminiscent of a Deposition from the Cross and gestures recalling Saint Thomas yet it is not intended to be a sacred artwork: it’s a mocking gesture upon entering such a strongly characterized space as the church – empty and stripped – the first thought is to restore its altarpiece with a touch of irony and gentle provocation installation view at Fondazione Sandra e Giancarlo Bonollo The dynamism and immersive dimension characterizing the two rooms hosting De Nisco’s canvases vanish when approaching the eleven works by Chiara Enzo presented in the exhibition “Fragments of Reality” Scattered across a space rendered aseptic by their fragmented presence these small images demand close observation making the «primary necessity of establishing a prolonged and draining contact with the scrutinized object…» which guides the artist’s creation and also the viewer’s experience as they strive to comprehend and reconstruct these entirely realistic but partial copies of reality What stands out in Enzo’s fragmentary frames intrigues from a narrative perspective How did the artist achieve such closeness to them (2021)’s face as he/she stretches his/her slender neck to the left?” The outcome of this close-range investigation is that the pastel-colored delicate yet vibrant surfaces end up mesmerizing those who trace the compositional lines of these fragments of worlds The unique texture created by layering different color shades on the paper’s grain conveys a hyper-realistic sensation of these epidermal fragments making viewers almost hear the heartbeat that pushed blood through the veins glimpsed beneath the depicted pale skin despite not knowing the represented subjects viewers end up feeling their presence vividly through associations with sensations experienced personally Milan and Fondazione Sandra e Giancarlo Bonollo The uniqueness of the Bonollo Foundation’s exhibition route ensures that the approach to art is entirely reversed from one exhibition to the other: first we are invited to partake in the rituals occurring in the misty atmospheres emanating from De Nisco’s large canvases from the familiar worlds spied upon through the keyholes provided by Enzo This performative way of experiencing contemporary art benefits the viewer granting absolute freedom in enjoying and engaging with the works in a comprehensive experience that leaves wide open the doors to countless unscripted dialogues between artworks and artists enhancing their communicative value for all audience levels If you are looking for an exceptional place to discover and rediscover contemporary art don’t miss visiting the Sandra and Giancarlo Bonollo Foundation in Thiene and following its program I am an art historian who grew up in the Asolo hills and who has always been immersed in contemporary art thanks to the family business of which I currently manage the Itaca Gallery exhibition space I consider art a polyphonic key to reading the reality that surrounds me and this cognitive value combined with the satisfaction I derive from its fruition ASSOCIAZIONE JULIET – via Battisti 19/a – 34015 Muggia (TS) Juliet art magazine è pubblicata a cura dell’Associazione Juliet - direttore responsabile Alessio Curto autorizzazione del Tribunale di Trieste registro informatico C.F./P.IVA 00699740320 | c/c postale 12103347 | SWIFT UNCRIT M10MC | IBAN IT75C0200802242000005111867 | UNICREDIT Banca Trieste The City of Vicenza acquired Palazzo Thiene on Monday September 6: the sixty days from the deed signed on June 10 have in fact passed without the State having exercised its right of first refusal; the City and Immobiliare Stampa signed the deed declaring the fulfillment of a condition precedent by which the transfer of ownership is concluded.After signing the drafted deed Vicenza Mayor Francesco Rucco and some representatives of the city council and council symbolically opened the doors of Palazzo Thiene “This is an achievement that the city administration has pursued with determination and stubbornness,” the mayor commented "because it was only right that this splendid Unesco heritage building former headquarters of Banca Popolare di Vicenza should become the property of the citizens our task is to make it known and enhance it also in relation to the candidacy of Vicenza as Italian City of Culture 2024 Within this month we will open to guided tours the museum part which at this stage will be managed by our civic museums in collaboration with the culture council committee we will consider whether to entrust it permanently to our structure which has all the credentials to take on this task or whether it will be more effective to activate external forms of management the Heritage department is already carrying out in-depth investigations on market values." It is very likely that the conception of the building is to be attributed to Giulio Romano (from 1523 in Mantua with the Gonzagas with whom the Thiene maintained very close relations) and that responsible for the executive design and construction of the building especially after Giulio Romano’s death in 1546 is the young Andrea Palladio When drug detection dog Rocky died suddenly due to GDV (Gastric Dilation-Volvulus) Many expressed their sympathies – including the very people Rocky was meant to help hunt down a message was found by the police station’s parking lot – with a small amount of drugs in it as ‘proof’ of its authenticity but we have feelings too,” the letter read Sadly we humans put animals in harm’s way for our own safety & wellbeing They love us so much they will do almost anything to please us That is one reason this type of news makes us all feel sad no matter what our profession If only humans were half as good/loyal and loving as your average dog we would all be better off We put dogs in needless harms way because some of us can’t seem to live their lives without harassing and attempting to control others even if it’s a war that can never be won and causing consequences that are much worse than the drugs themselves (I can speak from experience—10+ years IV heroin use and I’m still paying taxes Harry Potter fans will arrive from all over Italy to have a .. “If you have received the letter of admission you can enter and participate in the Hat Ceremony”: this is how the three days (and two nights) at the Italian School of Magic will begin.For the ’occasion the halls and rooms of the Thiene Castle will be themed with scenic decorations to immerse young guests in a magical and fantastic world among lessons in spells “The children who will participate,” says Francesca di Thiene and will have the opportunity to live for three days inside a real school of magic; those who have already attended the “School” in previous years will be able to continue their studies and obtain the title of apprentice magician year after year; places always sell out in very few days." The event includes an entertainment program run by professional actors “Our goal,” explains the Scuola di Magia Italiana team “is to transport participants to another world a world populated by wizards and witches and permeated with magic Each participant and staff member will wear an appropriate costume and impersonate a character h24 without ever stepping out of the part.” For 20 Milwaukee young people who grew up in low-income families the goal of becoming college graduates will become a dream fulfilled this spring in large part thanks to a program called College Possible Milwaukee works with high school students in 13 inner city high schools to support their college aspirations by offering weekly coaching sessions geared toward ACT preparation application completion and financial aid planning students continue to receive coaching support The result has been an 18% increase in ACT scores and a 97% college acceptance rate volunteers and donors to the program were present at the organization's "Dream Big" dinner April 23 at the University of Wisconsin-Milwaukee Jennifer and Tom Florsheim were honored for their early support of the College Possible program Among those there were Father Bill Johnson who is vice president for strategic growth at the new Cristo Rey Jesuit High School that will open this fall on Milwaukee's south side Father Johnson along with Michele Burke Deubel and Tina Quealy were representing the Burke Foundation that last year gave College Possible $700,000 Others seen at the dinner were Bruce and Helen Ambuel from the Mead-Witter Foundation and UWM Chancellor Mark Mone and wife Sara Swanson More College Possible students attend UWM than any other college although their numbers are represented at 80 colleges nationwide Also in attendance were Frank and Marina Krejci When you ask Barb Klebba where she lives the small town near Appleton where she used to reside she says "Kathy's House," the hospital guest house for families experiencing a medical crisis Klebba has been at the facility next to San Camillo in Wauwatosa for a year now while she receives treatment for stage 4 leukemia at Froedtert Hospital Living at the house has been a stress reliever for Klebba who doesn't have to worry about her living situation and can therefore devote more energy to fighting her cancer she has started a jewelry-making program for other residents and some of their wares were available for bid at the 15th annual Kathy's House Gala held April 25 at the Wisconsin Country Club Klebba was at the event with family members who cooks dinner for the Kathy's House residents four times a month donating his time as well as the groceries Also in attendance were Glen Van Fossen who brought his family son Greg and daughters Jill Wesp and Pam Nexa and a host of friends including Jim Strother And the mother and sisters of Kathy's House namesake Kathy Kuettner who died in 2000 from non-Hodgkins lymphoma too — Judy Vogel and daughters Karen Murray Those gathered at the Milwaukee Public Museum on April 25 to celebrate 35 years of Milwaukee Irish Fest felt like they were attending a family reunion with generations of musicians joined together to regale in fun times they shared on the Summerfest grounds since 1981 Irish Fest founder Ed Ward and his family were out in force including his daughters Kelly and Caitlin who also played in the Irish folk group Tallymoore that entertained the more than 400 revelers was there along with sister and brother-in-law Colleen and Tom Kennedy and their children Brian and Katie and sister-in-law Cathy Ward and her kids Bridget and Colin Also reuniting at the party were the original members of Blarney and regular Irish Fest performers in the party's early years John Testin and Kevin Stapleton who were there with their wives Stewart and Mary Oliver were there with and their daughter Emily and Elm Grove neighbors Cathy McVey John and Anita Steindorf and Trishand Rick Ormsby Wauwatosa mayor Kathy Ehley and her husband Kent and former Tosa mayor Maricolette Walsh and her husband Roger were there as were Eileen Carpenter Dave and Ann Kennedy,Linda Clark-Klibowitz and Roger Klibowitz Mary Ann Miller,Bill O'Toole and Dennis and Maureen Murphy Contact Helen Pauly by email at hpauly@journalsentinel.com Browse our weekly entertainment calendar listings from the Journal Sentinel Cue section Movie Listings: What's playing where this week The Italian clothing brand Aeronautica Militare celebrated its 20th anniversary in Florence with a party held within Pitti Uomo in the presence of industry insiders VIPs and several prominent military officials Photo: Aeronautica Militare Aeronautica Militare 20th anniversary celebrations The menswear brand was born in 2004 in Rivolto at the base of the Frecce Tricolori National Aerobatic Patrol as the first clothing collection featuring the same friezes badges and insignia that Italian Air Force pilots wear every day The Aeronautica Militare apparel brand is produced by the company Cristiano Di Thiene Spa under a licensing agreement since its birth The first collection made under this brand was inspired by real military uniforms and composed of 13 pieces including jackets Photo: Aeronautica Militare Aeronautica Militare celebrations Since then the bond between the company and the Italian Air Force Army has remained strong because the company is based in Thiene where there is an airport rich in history and witness to various missions including important battles fought by the Air Force during World War I Among the brand's most iconic pieces there is the leather jacket a symbol that brings to mind the early pioneers of flight who faced frigid temperatures at altitude protected only by a small windshield and the warmth of the clothing they wore the brand has continued to stand out and transformed itself through fashion trends by offering an increasingly wide-ranging collection consisting of technical and performance garments Photo: Aeronautica Militare Aeronautica Militare f/w 2024/2025 For f/w 2024/2025 the collection presented within the celebration event offers five product lines: Flying Team Within the Flying Team line,  they offer a special product capsule a selection of garments that pay tribute to the 91st Fighter Squadron known as the "Aces Squadron," and the protagonist of historic successful missions and distinguished by the Griffin symbol Each garment in this capsule is a tribute to aviation history and aims to capture the essence and courage of those glorious years and men The entire collection is distinguished by the application of patches but also high-performance technical fabrics and piece-dyed cottons Aeronautica Militare is sold through more than 900 stores 38 monobrand stores and 20 shops-in-the-shops Photo: Aeronautica Militare Aeronautica Militare f/w 2024/2025 The markets where brand is presently growing most are mainly Central and Eastern Europe $(document).ready(function() { adition.srq.push(function(api) { api.renderSlot("renderSlot_Rectangle-2"); }); }); READ ALSO: The Shows Seven historic residences in the Veneto region open their doors to offer the exclusive welcome of those who have lived in them for generations associating seven designers who will tell their design and creative journey through their objects and creations The Design in Villa festival will be held on the weekends of March 15-16 Villa da Schio and Villa Rosa.Organized by Dimore Amiche del Veneto under the patronage of theAssociazione Dimore Storiche Italiane Sezione Veneto the festival aims to create a bridge between art Six villas and a castle will open their doors to visitors; each dwelling will be associated with a designer will tell their design and creative path: Massimo Barbierato The meeting between these two realities stems from the common desire to enhance Italian craftsmanship a heritage of ancient workmanship that would risk being lost without the contribution of contemporary design Thanks to a wise balance between tradition and innovation the designers reinvent ancient workmanship transforming them into unique objects or produced in small series but handcrafted by the mastery of artisans The designer thus becomes the connecting point between past and future reinterpreting materials and techniques to bring to life creations that blend manual traditions and contemporary technologies Visiting the Dimore Amiche del Veneto during the festival means immersing oneself in a tale that spans more than 600 years of history visitors will be able to discover the stories of the Mansions and the works of the designers it will be possible to get to know them up close listen to their experiences and learn more about the link between their work and the Italian craft tradition Here is the detailed program Metrics details Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterised by progressive fibro-fatty replacement of right ventricular myocardium Earlier studies described ARVC as non-inflammatory non-coronary disorder associated with arrhythmias heart failure and sudden death due to functional exclusion of the right ventricle Molecular genetic studies have identified nine different loci associated with ARVC; accordingly each locus is implicated for each type of ARVC (ARVC1–ARVC9) So far five genes have been identified as containing pathogenic mutations for ARVC Though mutations in each of the gene/s indicate disruption of different pathways leading to the condition the exact pathogenesis of the condition is still obscure This review tries to understand the pathogenesis of the condition by examining the individual proteins implicated and relate them to the pathways that could play a role in the aetiology of the condition which regulates intra-cellular Ca2+ concentration by releasing Ca2+ reserves from the sarcoplasmic reticulum (SR) The mutation in this gene is believed to disrupt coupled gating of RYR-2 leading to arrhythmias followed by structural changes due to altered intra-cellular Ca2+ levels desmoplakin (ARVC8) and plakophilin (ARVC9) have prompted the speculation that ARVC is primarily a disease of desmosomes But identification of TGFβ-3 for ARVC1 and the role of all these three genes (plakoglobin desmoplakin and plakophilin) in cardiac morphogenesis indicate some kind of signal-transducing pathway disruption in the condition The finding that ARVC as a milder form of Uhl’s anomaly indicates similar ontogeny for the condition discovery of apoptotic cells in the autopsy of the right ventricular myocardium of ARVC patients does indicate a common pathway for different types of ARVCs which is more specific for the right ventricular myocardium involving desmosomal plaque proteins Heart failure is infrequent but may occur due to severe right or bi-ventricular enlargement heart failure and sudden death due to functional exclusion of the right ventricle among young people extensive clinical and molecular genetic studies have been carried out But the progress in clear understanding of the disorder has been impeded by the difficulty in diagnosis Electrocardiogram of an arrhythmogenic right ventricular cardiomyopathy (ARVC) patient showing right pre-cordial T–wave inversions Four clinicopathologic phases have been described based on the long-term follow-up of clinical data This phase results from the progression and extension of muscle disease with relatively preserved left ventricular function (Corrado et al. 1998) The final stage of bi-ventricular pump failure is caused by pronounced left ventricular involvement ARVC mimics biventricular dilated cardiomyopathy of other causes leading to congestive heart failure and related complications Various pathological and familial studies have described nine types of ARVC, the types being classified based on different loci mapped for the condition (Table 2) Nine different loci for the autosomal dominant form of ARVC indicate the underlying genetic heterogeneity five genes have been identified as containing the causative mutations for ARVC Other genes implicated in ARVC have to be identified in order to elucidate the pathogenic pathway leading to the condition FKBP thereby controls the opening of RYR2 in response to any trigger This process is very important for regulating the cytosolic [Ca2+] required for terminating the muscle contraction by reducing [Ca2+] required for relaxation and preventing diastolic depolarisation FKBP 12.6 binding makes RYR2 refractory to any trigger for Ca2+ release Schematic representation of desmosomal structure It can be concluded from the causative mutations for these disorders that absence of the segment of the tail region at the C terminal causes dilated cardiomyopathy the mutations causing the improper interactions with intermediate filaments or plakoglobin have been tolerated by the heart muscle and only complete absence of the segment or presence in only one copy would lead to heart abnormalities there is a ser299-to-arg (S299R) mis-sense mutation in exon 7 of the DSP gene This mutation is located in the N-terminal region modifying the putative PKC phosphorylation site in the minor heptad-rich Z region between the two alpha helical segments forming a loop that interacts with plakoglobin Unlike all the other desmoplakin related disorders there is no skin abnormality associated with the mutation in ARVC The epidermal tissue and the left ventricular myocardium seem to have tolerated the mutation This particular mutation seems to play a very important role in the right ventricular myocardium as it is inherited as autosomal dominant disorder The predilection for the right ventricle is yet to be understood There seems to be functional redundancy for C-terminal domain with some other proteins single dosage seems to be enough to show normal phenotype But manifestation of the N-terminal S299R in ARVC shows that no functional redundancy of the N-terminal globular domain is available in the right ventricular myocardial tissue Absence of keratoderma phenotype in ARVC can be explained by considering that only the mutations in C-terminal regions affect the skin phenotype The 2-bp deletion identified in Naxos eliminates nucleotides 2,157 and 2,158 causing shift in the reading frame and resulting in the premature termination and loss of 56 amino acids in the COOH– terminal end Since it is the –COOH end that is implicated in the signalling function the pathogenesis of ARVC may include some of the signalling activities disturbed along with the myocyte integrity The mice show lethal alterations in the morphogenesis and architecture of the heart with reduced trabeculation disarrayed cytoskeleton and rupture of cardiac walls absence of pkp2 also affects the desmosomal assembly desmoplakin dissociates from the desmosomal plaque and forms granular aggregates in the cytoplasm thereby affecting the connection of cardiomyocytes It is speculated that cell adhesion function is disrupted in cardiomyocytes as a result of mutations in plakophilin2 but the involvement of the signalling function of plakophilins in the pathogenesis cannot be ruled out TGFβ-3 is involved in the regulation of angiogenesis and trophoblast differentiation during hypoxia and plays a major role in cardiac morphogenesis TGFβ-3 has roles in mesenchyme differentiation development of fibrous septum primum of the atrium and fibrous skeleton of the heart The major role of TGFβ-3 in differentiation of the fibrous structures of the heart has recently been elucidated The twofold increase in TGFβ-3 synthesis may play a role in excess fibrosis (along with fatty replacement) observed in ARVC thus providing the basis for the trans-differentiation theory (discussed below) A foetal gene program must have been revoked in the adult heart leading to the replacement of the normal myocardium resulting in abnormal fibrous skeleton of the heart Arrhythmias observed in the case of ARVC could have been the result of these perturbations as the cardiac fibrous skeleton mainly serves in maintaining the electrophysiological discontinuity between the atria and ventricles (only connection via the atrioventricular bundle is allowed) Though the genes and causative mutations have been identified in the case of ARVC8 (Desmoplakin) the pathogenesis of the condition is obscure such as desmosomal plaque proteins (involved in cell adhesion) and ryanodine receptor (regulates intra-cellular Ca2+ concentration) and TGF (involved in morphogenesis and tissue remodelling) are implicated in causing different types of ARVC with similar histopathology and electrophysiology This indicates that a common pathway operates involving these proteins in early predilection to the right ventricle though all these genes except RYR-2 are expressed in many other tissues RYR-2 is not specific to the right ventricle alone but the pathway seems to involve the FKBP binding domain in conjunction with all the other proteins in the pathogenesis The implication of calcium channels and growth factors in the disease suggests that the mechanism may not be as simple as disruption of tissue integrity due to improper desmosomal plaque formation by the mutated proteins The mechanism of myocyte loss may be due to complex interactions specific to the right ventricle with many growth factors and intra-cellular ionic concentration playing a role Various theories have been proposed for explaining the aetiology of the condition which include: a congenital hypoplasia of the RV myocardium which presents in infancy as congestive heart failure (CHF) Uhl’s anomaly is characterised by partial or complete absence of the myocardium of the right ventricle The pathogenesis of the condition has not yet been elucidated but is believed to be due to incessant apoptosis progressively causing complete loss of the myocardium This may be the mechanism that operates in the case of Uhl’s anomaly where there is failure of cessation of apoptotic signals or anti-apoptotic signals fail to operate in order to rescue the right ventricular myocardium from complete loss The pathogenesis of ARVC also could involve a similar mechanism but the difference could be that in Uhl’s anomaly this process could end in complete destruction of the right ventricular myocardium this process would be episodic focal apoptosis beginning at any time in life and may re-occur The arrhythmias that appear in ARVC may be due to the cells undergoing apoptosis which may transiently exhibit increased excitability or the normal route of electrical signal transmission may be altered due to the apoptotic cells leading to micro-re-entrant or macro-entrant circuits An interesting observation was that the cells undergoing apoptosis were most frequently found in areas that have not undergone fibro-fatty replacement the apoptotic cells were observed in the areas already affected This shows that apoptosis could be the primary process that precedes fibro-fatty replacement of myocardial tissue Further elucidation of apoptosis as the primary process would require determining the triggering factors for apoptotic myocardial cell death If the theory of apoptotic post-natal morphogenesis is to be considered as involved in pathogenesis then the factors involved in this pathway are to be elucidated the question as to why apoptotic loss of the myocardium occurs intermittently in ARVC as opposed to progressive and complete loss in Uhl’s anomaly needs to be addressed developmental programmed cell death was observed which includes nuclear migration and extrusion This supports the hypothesis that a developmental gene program is revoked in this condition which would operate just after birth (for eliminating excess of right ventricular tissue) seems to have been revoked in ARVC at different intervals during the lifetime of the affected individual the trigger for ARVC is different from that of Uhl’s anomaly and the effect of this factor seems to be episodic a pathway needs to be elucidated that should include desmosomal plaque proteins such as plakoglobin calcium channel and the cardiac ryanodine receptor In all the cases where the genes have been identified RYR-2 deregulation due to mutations in the FKBP 12.6 binding domain could explain effort-induced arrhythmias the role of calcium signalling in apoptosis could be implicated in myocyte loss but the predilection of this loss to the right ventricle could not be explained Mutations in the C-terminal end of RYR-2 are known to cause catecholaminergic polymorphic ventricular tachycardia (CPVT) which includes arrhythmias without structural changes If the myocyte loss was only due to cytoplasmic calcium overload myocyte degeneration would have accompanied CPVT So some other pathway involving only the amino terminal domain would result in myocyte loss Mutation in the case of ARVC8 (S299R) in exon 7 of desmoplakin-modifying putative phosphorylation by PKC disrupts its interaction with plakoglobin This interaction may have another role in addition to the desmosomal assembly Plakoglobin and plakophilin have been directly demonstrated to have a role in morphogenesis of the heart it has to be further studied whether the Wnt pathway has any role in post-natal right ventricular morphogenesis The pathway involved in post-natal morphogenesis of the right ventricle should have desmosomes or desmosomal plaque proteins as its components with a trigger from growth factors such as TGFβ-3 and dependence on intra-cellular calcium concentration through interactions with amino terminal domain of the RYR-2 receptor Understanding the pathogenesis of this process would have instructional value in understanding additional mechanisms of developmental regulation of the heart apart from providing therapeutic significance Khaja F (1998) Arrhythmogenic right ventricular dysplasia/cardiomyopathy: review for the clinician Hughes AE (1999) Haploinsufficiency of desmoplakin causes a striate subtype of palmoplantar keratoderma Danieli GA (2000) Familial effort polymorphic ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy map to chromosome 1q42-q43 Rampazzo A (2005) Regulatory mutations in transforming growth factor-beta3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1 Kemler R (1999) Desmosomal localization of -catenin in the skin of plakoglobin null-mutant mice McKenna WJ (1998) Gene for arrhythmogenic right ventricular cardiomyopathy with diffuse nonepidermolytic palmoplantar keratoderma and woolly hair (Naxos disease) maps to 17q21 Pennelli N (1990) Sudden death in young competitive athletes: clinicopathologic correlation in 22 cases Thiene G (1998) Screening for hypertrophic cardiomyopathy in young athletes Gallo P (2000) Myocyte transdifferentiation: a possible pathogenetic mechanism for arrhythmogenic right ventricular cardiomyopathy Facquet J (1977) Stimulation studies and epicardial mapping in ventricular tachycardia: study of mechanisms and selection for surgery In: Kulbertus HE (eds) Reentrant Arrhythmias Fuchs E (1998) Desmoplakin is required early in development for assembly of desmosomes and cytoskeletal linkage Thierfelder L (2004) Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy Birchmeier W (2004) Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation Lopez SM (1996) Complete heart block and fatal right ventricular failure in an infant Franke WW (1988) Identification of a basic protein of Mr 75,000 as an accessory desmosomal plaque protein in stratified and complex epithelia Fontaine G (1996) Evidence of apoptosis in arrhythmogenic right ventricular dysplasia Marks AR (1998) Coupled gating between individual skeletal muscle Ca21 release channels (ryanodine receptors) Nava A et al (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy McKenna WJ (2000) Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease) Gittenberger-de-Groot AC (2003) Expression patterns of Tgfbeta1–3 associate with myocardialisation of the outflow tract and the development of the epicardium and the fibrous heart skeleton Thiene G (1988) Juvenile sudden death and effort induced ventricular tachycardias in a family with right ventricular cardiomyopathy Kelsell DP (2000) Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy Scampardonis G (1986) Cardiac abnormalities in familial palmoplantar keratosis Danieli GA (1995) A new locus for arrhythmogenic right ventricular cardiomyopathy(ARVD2)maps to chromosome 1q42-q43 Danieli GA (2002) Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy Franke WW (1996) Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart Johnson KR (1995) Identification of plakoglobin domains required for association with N-cadherin and a-catenin Pennelli N (1988) Right ventricular cardiomyopathy and sudden death in young people Rampazzo A (2001) Identification of mutations in cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2(ARVD 2) Tsukita S (1985) Desmocalmin: a calmodulin-binding high molecular weight protein isolated from desmosomes Rossi L (1998) Invivo evidence of apoptosis in arrhythmogenic right ventricular cardiomyopathy Vincent JP (1998) Signaling and adhesion activities of mammalian b-catenin and plakoglobin in Drosophila McGrath JA (2002) Compound heterozygosity for non-sense and mis-sense mutations in desmoplakin underlies skin fragility/woolly hair syndrome James TN (2000) Morphological patterns of death by myocytes in arrhythmogenic right ventricular dysplasia Download references Download citation DOI: https://doi.org/10.1007/s10038-005-0273-5 Anyone you share the following link with will be able to read this content: a shareable link is not currently available for this article Jan 13, 2025 | Airshow News, Display Team Schedules The Italian Aero Club has released a listing of airshows which will see participation by the Italian Air Force’s National Aerobatic Team All events listed below include full displays by the team unless noted is a global leader in the field of thermo printing and laminating With 50 years of engineering experience and over 10,000 customers in more than 70 countries the company plays an important role in technical textiles and various substrate bonding and coating Monti’s innovation has an oil heating patented system for hot melt cylinders It has incorporated this technology in the new generation Hot Melt Machines resulting in accurate even temperature controls reliable technology and enhanced safety for the operating personnel In his interaction with The Textile Magazine said: “The Indian market for hot melt is very promising and is still in the initial phase of growth and has great potential of diversifying its global sector production Indian customers are interested in new innovative technology and adding value to their business portfolio The Indian Government has also announced special benefits for the technical textile sector apart from the Technology Upgradation Fund Scheme (TUFS)” Monti is presenting new versions of hotmelt machinery as well as customizing a range of Indian clients Monti offers integrated solutions and local service support with its strategic partner Eastern Engineering Company in India The first one in the new range will be in production by end of 2013 The company’s 908 – hot melt modular provides maximum flexibility and productivity integrated in a single masterpiece machine The machine is a complete solution for customers willing to extend their business in coating and laminating with flexibility in two modules – full coating module and engraved cylinder module as per production needs The 914 all-in-one is an innovative concept for textile manufacturers who are eager to approach technical textile field in consideration of short-term return on investment and affordable solutions without compromising high-end quality This model has its focus on reliable heating technology with the pioneering concept of “built-in” glue distribution systems: engraved and full coating It has optimized working space and system of dynamic roller interchanging without losing dismantling time with effortless working and cleaning operations This model is composed of six different versions to have compatible budgetary solutions for all requirements These solutions include membrane entry system overfeeding centralizing web guide system and internal heating system without any external heating or boilers The official retail partner of the Italian Air Force Italian slow fashion brand… showcasing the best standards of Italian craftsmanship and style” Located on the third floor of St James Quarter the store design is a reflection of the Italian boutiques “incorporating soft tones and utilitarian fabrications for a minimal