title: Proteoglycans in cultures of skin fibroblasts in recessive dystrophic epidermolysis bullosa.
abstract: The proteoglycans of cultured fibroblasts from the skin of three patients with recessive dystrophic epidermolysis bullosa and three normals were compared after labeling with [35S]sulfate and [3H]leucine. The behavior in gel chromatography of the intact proteoglycans and several properties of their component glycosaminoglycans (size, content of iduronic acid, and content of 4- and 6-sulfate) showed no statistical differences. In addition, the binding of intact proteoglycans and of their constituent proteins and glycosaminoglycans to type I collagen were measured by affinity chromatography. No differences were found that could account for the skin lesions in recessive dystrophic epidermolysis bullosa.
authors: Rockefeller University, New York, NY 10021.
authors: J D
authors: JD
authors: Gregory
authors: J A
authors: JA
authors: Moy
authors: S P
authors: SP
authors: Damle
mesh_terms: D000293:Adolescent; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D002846:Chromatography, Affinity; D002847:Chromatography, Agarose; D016108:Epidermolysis Bullosa Dystrophica / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D005808:Genes, Recessive; D006025:Glycosaminoglycans / Q000737:chemistry / Q000378:metabolism; D006801:Humans; D011509:Proteoglycans / Q000378:metabolism*; D012016:Reference Values; D012867:Skin / Q000378:metabolism* / Q000473:pathology
chemical_list: D006025:Glycosaminoglycans; D011509:Proteoglycans