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Breakdown of the reciprocal stabilization of QBRICK/Frem1, Fras1, and Frem2 at the basement membrane provokes Fraser syndrome-like defects

An emerging family of extracellular matrix proteins characterized by 12 consecutive CSPG repeats and the presence of Calx-β motif(s) includes Fras1, QBRICK/Frem1, and Frem2. Mutations in the genes encoding these proteins have been associated with mouse models of Fraser syndrome, which is characteriz...

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Bibliografiske detaljer
Main Authors: Kiyozumi, Daiji, Sugimoto, Nagisa, Sekiguchi, Kiyotoshi
Format: Artigo
Sprog:Inglês
Udgivet: National Academy of Sciences 2006
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1567684/
https://ncbi.nlm.nih.gov/pubmed/16880404
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0601011103
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