Marfan syndrome: defective synthesis, secretion, and extracellular matrix formation of fibrillin by cultured dermal fibroblasts.

We studied the synthesis, secretion, and aggregation into the extracellular matrix of fibrillin by dermal fibroblasts from 26 probands with the Marfan syndrome. Cells from seven probands synthesized approximately half the normal amount of fibrillin when compared with intrafamilial or unrelated contr...

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Detalhes bibliográficos

Publicado no:	J Clin Invest
Principais autores:	Milewicz, D M, Pyeritz, R E, Crawford, E S, Byers, P H
Formato:	Artigo
Idioma:	Inglês
Publicado em:	American Society for Clinical Investigation 1992
Assuntos:	Research Article
Acesso em linha:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC442822/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/1729284/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI115589
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