Classical and Neonatal Marfan Syndrome Mutations in Fibrillin-1 Cause Differential Protease Susceptibilities and Protein Function

Mutations in fibrillin-1 give rise to Marfan syndrome (MFS) characterized by vascular, skeletal, and ocular abnormalities. Fibrillins form the backbone of extracellular matrix microfibrils in tissues including blood vessels, bone, and skin. They are crucial for regulating elastic fiber biogenesis an...

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Autores principales: Kirschner, Ryan, Hubmacher, Dirk, Iyengar, Garud, Kaur, Jasvir, Fagotto-Kaufmann, Christine, Brömme, Dieter, Bartels, Rainer, Reinhardt, Dieter P.
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Biochemistry and Molecular Biology 2011
Materias:
Glycobiology and Extracellular Matrices
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3173189/
https://ncbi.nlm.nih.gov/pubmed/21784848
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M111.221804
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