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Biochemical Changes in The Skin of Mink With Ehlers-Danlos Syndrome: Increased Collagen Biosynthesis in The Dermis of Affected Mink

1977; Elsevier BV; Volume: 69; Issue: 6 Linguagem: Inglês

10.1111/1523-1747.ep12687965

1523-1747

David F. Counts, Patricia Knighten, Gerald Α. Hegreberg,

Fibroblast Growth Factor Research

The Ehlers-Danlos syndromes (ED-S) are a group of connective tissue diseases which occur in humans and other mammals. Mink inherit an autosomal dominant form of ED-S which is characterized by laxity and decreased tensile strength of the skin. We wish to report some of the biochemical changes in the skin of affected mink as compared to age-matched, nonaffected mink. There was a 39% increase in acetic acid extractable collagen per wet weight of tissue of the skins of the affected mink. This was accompanied by a 260% increase in prolyl hydroxylase specific activity, a 179% increase in lysyl hydroxylase specific activity, and a 118% increase in lysyl oxidase specific activity in the skins of affected mink. [3H1-Hydroxyproline formation was increased 133% when skin tissue minces were incubated with [3H]-proline. This was accompanied by a 77% increase of [3H]-proline incorporation into protein and a 93% increase of [14C]-glycine incorporation into protein. Noncollagen protein synthesis, evaluated by measuring [3H]-tryptophane incorporation into protein, revealed a 40% increase in noncollagen protein synthesis. The increased collagen synthesis rate in the skins of the ED-S affected mink may represent either the absence of the control of collagen metabolism which contributes to the molecular defect of the ED-S in the affected mink, or a response to the damaged skin caused by the ED-S in the affected mink, or both.