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In the heart, fibrous networks of collagen fibers form throughout the ventricular myocardium and heart valves, and alterations in collagen fiber homeostasis are apparent in many forms of cardiac disease associated with myocardial dysfunction and valvular insufficiency. There is increasing evidence for cardiac dysfunction in connective tissue disorders, but the mechanisms have not been addressed. <jats:italic>ColVa1<\/jats:italic>+\/\u2212 and <jats:italic>ColXIa1<\/jats:italic>\u2212\/\u2212 mutant mice were used to identify roles for ColVa1 and ColXIa1 in ventricular myocardial morphogenesis and heart valve development. These affected cardiac structures show a compensatory increase in type I collagen deposition, similar to that previously described in valvular and cardiomyopathic disease. Morphological cardiac defects associated with changes in collagen fiber homeostasis identified in <jats:italic>ColVa1<\/jats:italic>+\/\u2212 and <jats:italic>ColXIa1<\/jats:italic>\u2212\/\u2212 mice provide an insight into previously unappreciated forms of cardiac dysfunction associated with connective tissue disorders. Developmental Dynamics 235:3295\u20133305, 2006. \u00a9 2006 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/dvdy.20980","type":"journal-article","created":{"date-parts":[[2006,10,6]],"date-time":"2006-10-06T19:28:50Z","timestamp":1160162930000},"page":"3295-3305","source":"Crossref","is-referenced-by-count":73,"title":["ColVa1 and ColXIa1 are required for myocardial morphogenesis and heart valve development"],"prefix":"10.1002","volume":"235","author":[{"given":"Joy","family":"Lincoln","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane B.","family":"Florer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gail H.","family":"Deutsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Wenstrup","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine 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