{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:09:19Z","timestamp":1790820559994,"version":"4.1.0"},"reference-count":45,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2019,8,1]],"date-time":"2019-08-01T00:00:00Z","timestamp":1564617600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,8,1]],"date-time":"2019-08-01T00:00:00Z","timestamp":1564617600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2018,11,14]],"date-time":"2018-11-14T00:00:00Z","timestamp":1542153600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["GGP11079"],"award-info":[{"award-number":["GGP11079"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011102","name":"European Commission Seventh Framework Programme","doi-asserted-by":"publisher","award":["602300"],"award-info":[{"award-number":["602300"]}],"id":[{"id":"10.13039\/100011102","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003407","name":"MIUR","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Matrix Biology"],"published-print":{"date-parts":[[2019,8]]},"DOI":"10.1016\/j.matbio.2018.11.002","type":"journal-article","created":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T18:40:33Z","timestamp":1542048033000},"page":"70-90","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":44,"special_numbering":"C","title":["Calcium activated nucleotidase 1 (CANT1) is critical for glycosaminoglycan biosynthesis in cartilage and endochondral ossification"],"prefix":"10.1016","volume":"81","author":[{"given":"Chiara","family":"Paganini","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Monti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossella","family":"Costantini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Besio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Lecci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3834-6712","authenticated-orcid":false,"given":"Marco","family":"Biggiogera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6170-7382","authenticated-orcid":false,"given":"Kun","family":"Tian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6472-0184","authenticated-orcid":false,"given":"Jean-Marc","family":"Schwartz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Huber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Cormier-Daire","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beth G.","family":"Gibson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katarzyna A.","family":"Pirog","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonella","family":"Forlino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Rossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.matbio.2018.11.002_bb0005","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1146\/annurev.cellbio.22.010605.093433","article-title":"The molecular diversity of glycosaminoglycans shapes animal development","volume":"22","author":"Bulow","year":"2006","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.matbio.2018.11.002_bb0010","doi-asserted-by":"crossref","first-page":"452315","DOI":"10.1155\/2014\/452315","article-title":"Metabolism of cartilage proteoglycans in health and disease","volume":"2014","author":"Vynios","year":"2014","journal-title":"Biomed. Res. Int."},{"key":"10.1016\/j.matbio.2018.11.002_bb0015","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.matbio.2015.02.003","article-title":"Proteoglycan form and function: a comprehensive nomenclature of proteoglycans","volume":"42","author":"Iozzo","year":"2015","journal-title":"Matrix Biol."},{"key":"10.1016\/j.matbio.2018.11.002_bb0020","series-title":"Extracellular Matrix: Pathobiology and Signaling, Walter de Gruyter, Berlin, Germany","first-page":"97","article-title":"Bone and skin disorders caused by a disturbance in the biosynthesis of chondroitin sulfate and dermatan sulfate","author":"Mizumoto","year":"2012"},{"issue":"495764","key":"10.1016\/j.matbio.2018.11.002_bb0025","first-page":"495764","article-title":"Human genetic disorders and knockout mice deficient in glycosaminoglycan","volume":"2014","author":"Mizumoto","year":"2014","journal-title":"Biomed. Res. Int."},{"issue":"12","key":"10.1016\/j.matbio.2018.11.002_bb0030","doi-asserted-by":"crossref","first-page":"2869","DOI":"10.1002\/ajmg.a.37365","article-title":"Nosology and classification of genetic skeletal disorders: 2015 revision","volume":"167","author":"Bonafe","year":"2015","journal-title":"Am. J. Med. Genet. A"},{"issue":"1","key":"10.1016\/j.matbio.2018.11.002_bb0035","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1002\/ajmg.a.20440","article-title":"Desbuquois dysplasia, a reevaluation with abnormal and \u201cnormal\u201d hands: radiographic manifestations","volume":"124A","author":"Faivre","year":"2004","journal-title":"Am. J. Med. Genet. A"},{"issue":"5","key":"10.1016\/j.matbio.2018.11.002_bb0040","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1016\/j.ajhg.2009.10.001","article-title":"Identification of CANT1 mutations in Desbuquois dysplasia","volume":"85","author":"Huber","year":"2009","journal-title":"Am. J. Hum. Genet."},{"issue":"3","key":"10.1016\/j.matbio.2018.11.002_bb0045","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/j.ajhg.2014.01.020","article-title":"XYLT1 mutations in Desbuquois dysplasia type 2","volume":"94","author":"Bui","year":"2014","journal-title":"Am. J. Hum. Genet."},{"issue":"40","key":"10.1016\/j.matbio.2018.11.002_bb0050","doi-asserted-by":"crossref","first-page":"36978","DOI":"10.1074\/jbc.M201656200","article-title":"Cloning, expression, and functional characterization of a Ca(2+)-dependent endoplasmic reticulum nucleoside diphosphatase","volume":"277","author":"Failer","year":"2002","journal-title":"J. Biol. Chem."},{"issue":"8","key":"10.1016\/j.matbio.2018.11.002_bb0055","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1002\/humu.22104","article-title":"Further delineation of CANT1 phenotypic spectrum and demonstration of its role in proteoglycan synthesis","volume":"33","author":"Nizon","year":"2012","journal-title":"Hum. Mutat."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0060","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1007\/s11302-005-5300-7","article-title":"The calcium activated nucleotidases: a diverse family of soluble and membrane associated nucleotide hydrolyzing enzymes","volume":"2","author":"Smith","year":"2006","journal-title":"Purinergic Signal"},{"issue":"1","key":"10.1016\/j.matbio.2018.11.002_bb0065","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/S0003-9861(02)00420-4","article-title":"Cloning, expression, and characterization of a soluble calcium-activated nucleotidase, a human enzyme belonging to a new family of extracellular nucleotidases","volume":"406","author":"Smith","year":"2002","journal-title":"Arch. Biochem. Biophys."},{"issue":"22","key":"10.1016\/j.matbio.2018.11.002_bb0070","doi-asserted-by":"crossref","first-page":"18478","DOI":"10.1074\/jbc.M111.304733","article-title":"Ca2+\u2212activated nucleotidase 1, a novel target gene for the transcriptional repressor DREAM (downstream regulatory element antagonist modulator), is involved in protein folding and degradation","volume":"287","author":"Cali","year":"2012","journal-title":"J. Biol. Chem."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0075","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1111\/j.1748-1716.2010.02116.x","article-title":"Signalling and pharmacological properties of the P2Y receptor","volume":"199","author":"Harden","year":"2010","journal-title":"Acta Physiol (Oxford)"},{"key":"10.1016\/j.matbio.2018.11.002_bb0080","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.matbio.2018.03.004","article-title":"Paradoxical roles of ATF6\u03b1 and ATF6\u03b2 in modulating disease severity caused by mutations in collagen X","volume":"70","author":"Forouhan","year":"2018","journal-title":"Matrix Biol."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0085","doi-asserted-by":"crossref","first-page":"162","DOI":"10.2106\/00004623-198769020-00002","article-title":"Quantitation of chondrocyte performance in growth-plate cartilage during longitudinal bone growth","volume":"69","author":"Hunziker","year":"1987","journal-title":"J. Bone Joint Surg. Am."},{"issue":"6","key":"10.1016\/j.matbio.2018.11.002_bb0090","doi-asserted-by":"crossref","first-page":"1483","DOI":"10.3892\/ijmm.2015.2158","article-title":"The utility of mouse models to provide information regarding the pathomolecular mechanisms in human genetic skeletal diseases: the emerging role of endoplasmic reticulum stress (review)","volume":"35","author":"Briggs","year":"2015","journal-title":"Int. J. Mol. Med."},{"key":"10.1016\/j.matbio.2018.11.002_bb0095","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/j.matbio.2018.05.005","article-title":"Cartilage diseases","volume":"71-72","author":"Krishnan","year":"2018","journal-title":"Matrix Biol."},{"issue":"7","key":"10.1016\/j.matbio.2018.11.002_bb0100","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1002\/dvdy.24131","article-title":"Mechanisms and models of endoplasmic reticulum stress in chondrodysplasia","volume":"243","author":"Patterson","year":"2014","journal-title":"Dev. Dyn."},{"issue":"6","key":"10.1016\/j.matbio.2018.11.002_bb0105","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.matbio.2010.05.001","article-title":"Defective proteoglycan sulfation of the growth plate zones causes reduced chondrocyte proliferation via an altered Indian hedgehog signalling","volume":"29","author":"Gualeni","year":"2010","journal-title":"Matrix Biol."},{"issue":"11","key":"10.1016\/j.matbio.2018.11.002_bb0110","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1038\/nm1655","article-title":"Nucleotide-sugar transporter SLC35D1 is critical to chondroitin sulfate synthesis in cartilage and skeletal development in mouse and human","volume":"13","author":"Hiraoka","year":"2007","journal-title":"Nat. Med."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0115","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.ydbio.2012.01.005","article-title":"Chondroitin sulfate synthase 1 (Chsy1) is required for bone development and digit patterning","volume":"363","author":"Wilson","year":"2012","journal-title":"Dev. Biol."},{"issue":"17","key":"10.1016\/j.matbio.2018.11.002_bb0120","doi-asserted-by":"crossref","first-page":"3989","DOI":"10.1242\/dev.01948","article-title":"Maintenance of chondroitin sulfation balance by chondroitin-4-sulfotransferase 1 is required for chondrocyte development and growth factor signaling during cartilage morphogenesis","volume":"132","author":"Kluppel","year":"2005","journal-title":"Development"},{"issue":"4","key":"10.1016\/j.matbio.2018.11.002_bb0125","doi-asserted-by":"crossref","first-page":"57R","DOI":"10.1093\/glycob\/12.4.57R","article-title":"Chondrodysplasias due to proteoglycan defects","volume":"12","author":"Schwartz","year":"2002","journal-title":"Glycobiology"},{"key":"10.1016\/j.matbio.2018.11.002_bb0130","doi-asserted-by":"crossref","DOI":"10.1038\/nrdp.2017.52","article-title":"Osteogenesis imperfecta","volume":"3","author":"Marini","year":"2017","journal-title":"Nat Rev Dis Primers"},{"key":"10.1016\/j.matbio.2018.11.002_bb0135","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/j.matbio.2018.03.010","article-title":"Osteogenesis imperfecta and therapeutics","volume":"71\u201372","author":"Morello","year":"2018","journal-title":"Matrix Biol."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0140","first-page":"291","article-title":"BiP: master regulator of the unfolded protein response and crucial factor in Flavivirus biology","volume":"90","author":"Lewy","year":"2017","journal-title":"Yale J Biol Med"},{"issue":"8","key":"10.1016\/j.matbio.2018.11.002_bb0145","doi-asserted-by":"crossref","first-page":"1608","DOI":"10.1002\/jbmr.2824","article-title":"Osteoblast malfunction caused by cell stress response to procollagen Misfolding in \u03b12(I)-G610C mouse model of osteogenesis imperfecta","volume":"31","author":"Mirigian","year":"2016","journal-title":"J. Bone Miner. Res."},{"issue":"42","key":"10.1016\/j.matbio.2018.11.002_bb0150","doi-asserted-by":"crossref","first-page":"16171","DOI":"10.1073\/pnas.0804259105","article-title":"Dyggve-Melchior-Clausen syndrome: chondrodysplasia resulting from defects in intracellular vesicle traffic","volume":"105","author":"Osipovich","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"6102","key":"10.1016\/j.matbio.2018.11.002_bb0155","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1126\/science.1224947","article-title":"Sedlin controls the ER export of procollagen by regulating the Sar1 cycle","volume":"337","author":"Venditti","year":"2012","journal-title":"Science"},{"issue":"6","key":"10.1016\/j.matbio.2018.11.002_bb0160","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.1086\/320594","article-title":"A recurrent RNA-splicing mutation in the SEDL gene causes X-linked spondyloepiphyseal dysplasia tarda","volume":"68","author":"Tiller","year":"2001","journal-title":"Am. J. Hum. Genet."},{"issue":"1","key":"10.1016\/j.matbio.2018.11.002_bb0165","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1038\/sj.cr.7290106","article-title":"Structure and function of aggrecan","volume":"12","author":"Kiani","year":"2002","journal-title":"Cell Res."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0170","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.ydbio.2009.02.024","article-title":"Aggrecan modulation of growth plate morphogenesis","volume":"329","author":"Domowicz","year":"2009","journal-title":"Dev. Biol."},{"issue":"6","key":"10.1016\/j.matbio.2018.11.002_bb0175","first-page":"1414","article-title":"A novel transgenic mouse model of growth plate dysplasia reveals that decreased chondrocyte proliferation due to chronic ER stress is a key factor in reduced bone growth","volume":"6","author":"Gualeni","year":"2013","journal-title":"Dis. Model. Mech."},{"issue":"2","key":"10.1016\/j.matbio.2018.11.002_bb0180","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/j.cell.2006.08.039","article-title":"CRTAP is required for prolyl 3-hydroxylation and mutations cause recessive osteogenesis imperfecta","volume":"127","author":"Morello","year":"2006","journal-title":"Cell"},{"issue":"4","key":"10.1016\/j.matbio.2018.11.002_bb0185","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1016\/S1357-2725(02)00301-1","article-title":"The chondrocyte","volume":"35","author":"Archer","year":"2003","journal-title":"Int. J. Biochem. Cell Biol."},{"issue":"6","key":"10.1016\/j.matbio.2018.11.002_bb0190","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1093\/hmg\/ddi079","article-title":"A diastrophic dysplasia sulfate transporter (SLC26A2) mutant mouse: morphological and biochemical characterization of the resulting chondrodysplasia phenotype","volume":"14","author":"Forlino","year":"2005","journal-title":"Hum. Mol. Genet."},{"issue":"4","key":"10.1016\/j.matbio.2018.11.002_bb0195","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/S8756-3282(97)00286-X","article-title":"Kinetic studies on epiphyseal growth cartilage in the normal mouse","volume":"22","author":"Vanky","year":"1998","journal-title":"Bone"},{"issue":"5","key":"10.1016\/j.matbio.2018.11.002_bb0200","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/S0945-053X(98)90088-9","article-title":"Proteoglycan sulfation in cartilage and cell cultures from patients with sulfate transporter chondrodysplasias: relationship to clinical severity and indications on the role of intracellular sulfate production","volume":"17","author":"Rossi","year":"1998","journal-title":"Matrix Biol."},{"issue":"1","key":"10.1016\/j.matbio.2018.11.002_bb0205","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1042\/bj2720193","article-title":"Independent secretion of proteoglycans and collagens in chick chondrocyte cultures during acute ascorbic acid treatment","volume":"272","author":"Pacifici","year":"1990","journal-title":"Biochem. J."},{"issue":"1\u20132","key":"10.1016\/j.matbio.2018.11.002_bb0210","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1016\/j.cca.2006.11.025","article-title":"Reliability of 1,9-dimethylmethylene blue tests in comparison to agarose gel electrophoresis for quantification of urinary glycosaminoglycans","volume":"378","author":"de Lima","year":"2007","journal-title":"Clin. Chim. Acta"},{"issue":"19","key":"10.1016\/j.matbio.2018.11.002_bb0215","doi-asserted-by":"crossref","first-page":"5570","DOI":"10.1093\/hmg\/ddv289","article-title":"N-acetylcysteine treatment ameliorates the skeletal phenotype of a mouse model of diastrophic dysplasia","volume":"24","author":"Monti","year":"2015","journal-title":"Hum. Mol. Genet."},{"issue":"6","key":"10.1016\/j.matbio.2018.11.002_bb0220","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1177\/31.6.6341460","article-title":"Ruthenium hexammine trichloride (RHT)-mediated interaction between plasmalemmal components and pericellular matrix proteoglycans is responsible for the preservation of chondrocytic plasma membranes in situ during cartilage fixation","volume":"31","author":"Hunziker","year":"1983","journal-title":"J. Histochem. Cytochem."},{"issue":"5 Pt A","key":"10.1016\/j.matbio.2018.11.002_bb0225","doi-asserted-by":"crossref","first-page":"1642","DOI":"10.1016\/j.bbadis.2018.02.002","article-title":"4-PBA ameliorates cellular homeostasis in fibroblasts from osteogenesis imperfecta patients by enhancing autophagy and stimulating protein secretion","volume":"1864","author":"Besio","year":"2018","journal-title":"Biochim. Biophys. Acta, Mol. Basis Dis."}],"container-title":["Matrix Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0945053X18303974?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0945053X18303974?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,15]],"date-time":"2025-10-15T18:58:18Z","timestamp":1760554698000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0945053X18303974"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,8]]},"references-count":45,"alternative-id":["S0945053X18303974"],"URL":"https:\/\/doi.org\/10.1016\/j.matbio.2018.11.002","relation":{},"ISSN":["0945-053X"],"issn-type":[{"value":"0945-053X","type":"print"}],"subject":[],"published":{"date-parts":[[2019,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Calcium activated nucleotidase 1 (CANT1) is critical for glycosaminoglycan biosynthesis in cartilage and endochondral ossification","name":"articletitle","label":"Article Title"},{"value":"Matrix Biology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.matbio.2018.11.002","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 The Authors. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}