The structural basis of cystic fibrosis

Xin Meng; Jack Clews; Eleanor R. Martin; Anca D. Ciuta; Robert C. Ford

doi:10.1042/BST20180296

The structural basis of cystic fibrosis

Xin Meng, Jack Clews, Eleanor R. Martin, Anca D. Ciuta, Robert C. Ford^*

^*Corresponding author for this work

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Research output: Contribution to journal › Review article › peer-review

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.

Original language	English
Pages (from-to)	1093-1098
Number of pages	6
Journal	Biochemical Society Transactions
Volume	46
Issue number	5
Early online date	28 Aug 2018
DOIs	https://doi.org/10.1042/BST20180296
Publication status	Published - 2018

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10.1042/BST20180296

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title = "The structural basis of cystic fibrosis",

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.",

author = "Xin Meng and Jack Clews and Martin, {Eleanor R.} and Ciuta, {Anca D.} and Ford, {Robert C.}",

year = "2018",

doi = "10.1042/BST20180296",

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volume = "46",

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AU - Meng, Xin

AU - Clews, Jack

AU - Martin, Eleanor R.

AU - Ciuta, Anca D.

AU - Ford, Robert C.

PY - 2018

Y1 - 2018

N2 - 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.

AB - 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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M3 - Review article

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SN - 0300-5127

VL - 46

SP - 1093

EP - 1098

JO - Biochemical Society Transactions

JF - Biochemical Society Transactions

IS - 5

ER -

The structural basis of cystic fibrosis

Abstract

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