A heterozygous defect for structurally altered pro-alpha 2 chain of type I procollagen in a mild variant of osteogenesis imperfecta. The altered structure decreases the thermal stability of procollagen and makes it resistant to procollagen N-proteinase.-Reference-Cited by-同舟云学术

A heterozygous defect for structurally altered pro-alpha 2 chain of type I procollagen in a mild variant of osteogenesis imperfecta. The altered structure decreases the thermal stability of procollagen and makes it resistant to procollagen N-proteinase.

Published:1984-11 Issue:22 Volume:259 Page:14094-14100
ISSN:0021-9258
Container-title:Journal of Biological Chemistry
language:en
Short-container-title:Journal of Biological Chemistry

Author:

Sippola M,Kaffe S,Prockop D J

Publisher

Elsevier BV

Subject

Cell Biology,Molecular Biology,Biochemistry

Reference34 articles.

1. Metabolic Basis of Heritable Disorders, fifth edition;Pinnell,1983

2. The Brittle Bone Syndrome, Osteogenesis Imperfecta;Smith,1983

3. Heritable Diseases of Collagen

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