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Genetic defects affecting the biosynthesis and/or structure of the fibrillar collagens have been implicated in the classic (type V collagen, OMIM 130000), vascular (type III collagen, OMIM 130050), arthrochalasia (type I collagen, OMIM 130060), kyphoscoliosis (lysylhydroxylase 1, OMIM 225400), and dermatosparaxis (ADAMTS-2, OMIM 225410) forms of EDS

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