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scientific articles

Patients with Ehlers-Danlos Syndrome Type IV Lack Type III Collagen

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source: Proceedings of the National Academy of Sciences of the United States of America

year: 1975

authors: Pope FM, Martin GR, Lichtenstein JR, Penttinen R, Gerson B, Rowe DW, McKusick VA

summary/abstract:

One of the genetically distinct collagens (type III) normally found in skin, aorta, and intestine is missing from the tissues of patients with the Ehlers-Danlos syndrome type IV. While skin fibroblasts from other individuals synthesize both types I and III collagen. Ehlers-Danlos syndrome IV cells synthesize only type I. These results suggest that the fragile skin, blood vessels, and intestines of Ehlers-Danlos syndrome IV patients result from an absence of type III collagen.

organization: National Institute of Dental Research Bethesda; Johns Hopkins Hospital; Armed Forces Institute of Pathology

DOI: 10.1073/pnas.72.4.1314

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