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Assembly assay identifies a critical region of human fibrillin-1 required for 10–12 nm diameter microfibril biogenesis

The human FBN1 gene encodes fibrillin-1 (FBN1); the main component of the 10–12 nm diameter extracellular matrix microfibrils. Marfan syndrome (MFS) is a common inherited connective tissue disorder, caused by FBN1 mutations. It features a wide spectrum of disease severity, from mild cases to the let...

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Bibliografiske detaljer
Udgivet i:PLoS One
Main Authors: Jensen, Sacha A., Atwa, Ondine, Handford, Penny A.
Format: Artigo
Sprog:Inglês
Udgivet: Public Library of Science 2021
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC7971562/
https://ncbi.nlm.nih.gov/pubmed/33735269
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0248532
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