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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Veröffentlicht in:	J Clin Invest
Hauptverfasser:	Milewicz, D M, Pyeritz, R E, Crawford, E S, Byers, P H
Format:	Artigo
Sprache:	Inglês
Veröffentlicht:	American Society for Clinical Investigation 1992
Schlagworte:	Research Article
Online-Zugang:	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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