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Cysteine Substitutions in Epidermal Growth Factor–Like Domains of Fibrillin-1: Distinct Effects on Biochemical and Clinical Phenotypes

Fibrillin-1 (FBN1) contains 47 epidermal growth factor (EGF)–like domains characterized by six conserved cysteine residues. Cysteine substitutions that disrupt one of the three disulfide bonds are frequent causes of Marfan syndrome (MFS). We identified 19 new substitutions involving cysteine residue...

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Detalhes bibliográficos
Main Authors: Schrijver, I., Liu, W., Brenn, T., Furthmayr, H., Francke, U.
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society of Human Genetics 1999
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1288233/
https://ncbi.nlm.nih.gov/pubmed/10486319
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