title: [Type VII C Ehlers-Danlos disease or human dermatosparaxia: the product of a fruitful union of so-called fundamental sciences with human and veterinary clinical medicine].
abstract: Ehlers-Danlos type VII C disease is a heritable disorder of the connective tissues and a disease similar to dermatosparaxia in bovine and other animal species. The determination of the pathomechanism of these diseases, the inactivity of the specific protease excising the aminoterminal extension of the type I collagen precursor, resulted of interactive basic and clinical research.
authors.affiliation: Service de Dermatologie, CHU du Sart Tilman, Université de Liège.
authors.forename: C M
authors.initials: CM
authors.lastname: Lapière
authors.forename: B V
authors.initials: BV
authors.lastname: Nusgens
mesh_terms: D004535:Ehlers-Danlos Syndrome / Q000145:classification / Q000201:enzymology / Q000235:genetics*; D005799:Genes, Dominant; D006801:Humans; D007593:Joint Instability / Q000235:genetics; D011348:Procollagen N-Endopeptidase / Q000378:metabolism
chemical_list: D011348:Procollagen N-Endopeptidase
vernacular_title: La maladie d'Ehlers-Danlos de type VII C ou dermatosparaxie humaine: le produit d'une union fructueuse des sciences dites fondamentales avec la clinique humaine et vétérinaire.