学術雑誌論文

学術雑誌論文

text

Journal of Cell Biology. 2012, 197(5), p. 677-689

Basement membrane assembly of the integrin α8β1 ligand nephronectin requires Fraser syndrome–associated proteins

Rockefeller University Press

英語

text

2012-05

https://doi.org/10.1083/jcb.201203065

©2012 Kiyozumi et al. Originally published in the Journal of Cell Biology. https://doi.org/10.1083/jcb.201203065

Dysfunction of the basement membrane protein QBRICK provokes Fraser syndrome, which results in renal dysmorphogenesis, cryptophthalmos, syndactyly, and dystrophic epidermolysis bullosa through unknown mechanisms. Here, we show that integrin α8β1 binding to basement membranes was significantly impaired in Qbrick-null mice. This impaired integrin α8β1 binding was not a direct consequence of the loss of QBRICK, which itself is a ligand of integrin α8β1, because knock-in mice with a mutation in the integrin-binding site of QBRICK developed normally and do not exhibit any defects in integrin α8β1 binding. Instead, the loss of QBRICK significantly diminished the expression of nephronectin, an integrin α8β1 ligand necessary for renal development. In vivo, nephronectin associated with QBRICK and localized at the sublamina densa region, where QBRICK was also located. Collectively, these findings indicate that QBRICK facilitates the integrin α8β1–dependent interactions of cells with basement membranes by regulating the basement membrane assembly of nephronectin and explain why renal defects occur in Fraser syndrome.

71818

Basement membrane assembly of the integrin α8β1 ligand nephronectin requires Fraser syndrome–associated proteins

Rockefeller University Press

英語

2012-05

Journal of Cell Biology

197

5

677

689

https://doi.org/10.1083/jcb.201203065

https://hdl.handle.net/11094/71818