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Abnormal fibrillin assembly by dermal fibroblasts from two patients with Marfan syndrome

The microfibrillar glycoprotein fibrillin is linked to the Marfan syndrome, an autosomal dominant connective tissue disorder. In this study, fibrillin synthesis, deposition and assembly has been investigated in Marfan dermal fibroblast lines from two unrelated patients for whom distinct mutations in...

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Natura: Artigo
Lingua:Inglês
Pubblicazione: The Rockefeller University Press 1994
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2119967/
https://ncbi.nlm.nih.gov/pubmed/8132720
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