Patients with Ehlers-Danlos syndrome type IV lack type III collagen. | Zendy

F M Pope | Zendy; George R. Martin | Zendy; J R Lichtenstein | Zendy; Risto Penttinen | Zendy; B Gerson | Zendy; David W. Rowe | Zendy; V.A. McKusick | Zendy

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Patients with Ehlers-Danlos syndrome type IV lack type III collagen.

Author(s) -

F M Pope,

George R. Martin,

J R Lichtenstein,

Risto Penttinen,

B Gerson,

David W. Rowe,

V.A. McKusick

Publication year - 1975

Publication title -

proceedings of the national academy of sciences

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 5.011

H-Index - 771

eISSN - 1091-6490

pISSN - 0027-8424

DOI - 10.1073/pnas.72.4.1314

Subject(s) - ehlers–danlos syndrome , type i collagen , collagen, type i, alpha 1 , aorta , pathology , chemistry , medicine , biochemistry , extracellular matrix

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.

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