ALTERED SECRETION OF TYPE-III PROCOLLAGEN IN A FORM OF TYPE-IV EHLERS-DANLOS SYNDROME - BIOCHEMICAL-STUDIES IN CULTURED FIBROBLASTS
- 1 January 1981
- journal article
- research article
- Vol. 44 (4), 336-341
Abstract
Cultured dermal fibroblasts from a woman with 1 variety of type IV Ehlers-Danlos syndrome synthesize type III procollagen but fail to secrete the bulk of the protein. Although total collagen production is similar to that of controls, the affected cells retain almost twice as much collagen as controls. The additional retained protein is a disulfide-bonded collagenous trimer that remains disulfide-linked after limited proteolysis with pepsin and, after pepsin treatment, migrates with type III collagen on polyacrylamide gel electrophoresis. Affected cells have markedly increased staining with antibodies directed against type III procollagen. Apparently, decreased secretion of the type III procollagen that is synthesized can result in the clinical syndrome of type IV Ehlers-Danlos syndrome.This publication has 9 references indexed in Scilit:
- ULTRASTRUCTURAL CHARACTERISTICS OF THE SKIN IN A FORM OF THE EHLERS-DANLOS SYNDROME TYPE-IV - STORAGE IN THE ROUGH ENDOPLASMIC-RETICULUM1981
- Effects of tunicamycin on the biosynthesis of procollagen by human fibroblasts.Journal of Biological Chemistry, 1980
- Clinical and ultrastructural heterogeneity of type IV Ehlers-Danlos syndromeHuman Genetics, 1979
- Correlation of procollagen mRNA levels in normal and transformed chick embryo fibroblasts with different rates of procollagen synthesisBiochemistry, 1978
- Cell surface-associated structural proteins in connective tissue cells.Proceedings of the National Academy of Sciences, 1977
- Inheritance of Ehlers-Danlos type IV syndrome.Journal of Medical Genetics, 1977
- Impaired conversion of procollagen to collagen by fibroblasts and bone treated with tunicamycin, an inhibitor of protein glycosylation.Journal of Biological Chemistry, 1977
- Simultaneous synthesis of types I and III collagen by fibroblasts in culture.Proceedings of the National Academy of Sciences, 1976
- The estimation of two collagens from human dermis by interrupted gel electrophoresisBiochemical and Biophysical Research Communications, 1976