A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator

FEBS Letters
J P AnnereauV Stoven

Abstract

Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The most frequent mutation is the deletion of F508 in the first nucleotide binding fold (NBF1). It induces a perturbation in the folding of NBF1, which impedes posttranslational maturation of CFTR. Determination of the three-dimensional structure of NBF1 would help to understand this defect. We present a novel model for NBF1 built from the crystal structure of bovine mitochondrial F1-ATPase protein. This model gives a reasonable interpretation of the effect of mutations on the maturation of the protein and, in agreement with the CD data, leads to reconsideration of the limits of NBF1 within CFTR.

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Citations

Oct 7, 2004·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Anne VankeerberghenJean-Jacques Cassiman
Apr 22, 1999·Protein Science : a Publication of the Protein Society·A A SalamovM B Swindells
Apr 29, 1998·Annual Review of Physiology·J K Foskett
May 20, 1999·The Journal of Clinical Investigation·N KälinB Tümmler
Apr 29, 1999·Proceedings of the National Academy of Sciences of the United States of America·R SchreiberK Kunzelmann
Oct 5, 2007·Proceedings of the National Academy of Sciences of the United States of America·Toby S Scott-WardA Christopher Boyd
Sep 26, 2000·Molecular Genetics and Metabolism·J M ChenC Ferec
Mar 23, 2011·Molecular Cell·Amardeep KhushooWilliam R Skach
Apr 17, 2014·The International Journal of Biochemistry & Cell Biology·Norbert Odolczyk, Piotr Zielenkiewicz
Nov 24, 1999·Biochimica Et Biophysica Acta·J F PolletP Jacobs
Jan 29, 1999·Physiological Reviews·D N Sheppard, M J Welsh
Jan 29, 1999·Physiological Reviews·D C Gadsby, A C Nairn
Apr 5, 2000·Biochimica Et Biophysica Acta·F L Theodoulou

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