Kniest dysplasia is characterized by an apparent abnormal processing of the C-propeptide of type II cartilage collagen resulting in imperfect fibril assembly.

Epiphyseal and growth plate cartilages from four cases of Kniest dysplasia have been studied. In each case collagen fibril organization appeared abnormal by electron microscopy compared with age-matched normal cartilages: fibrils were much thinner, of irregular shape and did not exhibit the characte...

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Detalhes bibliográficos
Main Authors: Poole, A R, Pidoux, I, Reiner, A, Rosenberg, L, Hollister, D, Murray, L, Rimoin, D
Formato: Artigo
Idioma:Inglês
Publicado em: 1988
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC329606/
https://ncbi.nlm.nih.gov/pubmed/3276736
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