The structural basis of cystic fibrosis

Biochemical Society Transactions
Xin MengRobert C Ford

Abstract

CFTR (ABCC7) is a phospho-regulated chloride channel that is found in the apical membranes of epithelial cells, is gated by ATP and the activity of the protein is crucial in the homeostasis of the extracellular liquid layer in many organs [Annu. Rev. Biochem. (2008) 77, 701-726; Science (1989) 245, 1066-1073]. Mutations in CFTR cause the inherited disease cystic fibrosis (CF), the most common inherited condition in humans of European descent [Science (1989) 245, 1066-1073; Pflugers Arch. (2007) 453, 555-567]. The structural basis of CF will be discussed in this article.

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Citations

Feb 10, 2019·Biological Chemistry·Xin MengRobert C Ford
Feb 15, 2019·Minerva pediatrica·Valeria R VillellaLuigi Maiuri
Jul 22, 2019·Cellular and Molecular Life Sciences : CMLS·Bianka FarkasTamás Hegedűs
Jan 11, 2019·Biochemical Society Transactions·Robert C Ford, Konstantinos Beis
Sep 4, 2020·FEBS Letters·Robert C Ford, Ute A Hellmich
Jun 3, 2021·International Journal of Molecular Sciences·Valeria CapurroNicoletta Pedemonte

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