Detalhe da pesquisa
1.
The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation.
Proteomics
; 15(2-3): 447-61, 2015 Jan.
Artigo
em Inglês
| MEDLINE | ID: mdl-25330774
2.
Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention.
Genet Med
; 16(8): 625-32, 2014 Aug.
Artigo
em Inglês
| MEDLINE | ID: mdl-24556927
3.
Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5.
Biochem J
; 452(3): 391-400, 2013 Jun 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-23566014
4.
Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells.
bioRxiv
; 2024 Mar 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-38496440
5.
Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.
Ann Neurol
; 67(6): 802-8, 2010 Jun.
Artigo
em Inglês
| MEDLINE | ID: mdl-20517942
6.
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator.
iScience
; 24(6): 102542, 2021 Jun 25.
Artigo
em Inglês
| MEDLINE | ID: mdl-34142049
7.
A chemical corrector modifies the channel function of F508del-CFTR.
Mol Pharmacol
; 78(3): 411-8, 2010 Sep.
Artigo
em Inglês
| MEDLINE | ID: mdl-20501743
8.
The intact CFTR protein mediates ATPase rather than adenylate kinase activity.
Biochem J
; 412(2): 315-21, 2008 Jun 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-18241200
9.
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.
J Cyst Fibros
; 18(1): 35-43, 2019 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-29685812
10.
ATP depletion inhibits the endocytosis of ClC-2.
J Cell Physiol
; 214(1): 273-80, 2008 Jan.
Artigo
em Inglês
| MEDLINE | ID: mdl-17620322
11.
The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer.
Biochem J
; 401(2): 581-6, 2007 Jan 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-16989640
12.
Nucleotides bind to the C-terminus of ClC-5.
Biochem J
; 398(2): 289-94, 2006 Sep 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-16686597
13.
Orkambi® and amplifier co-therapy improves function from a rare CFTR mutation in gene-edited cells and patient tissue.
EMBO Mol Med
; 9(9): 1224-1243, 2017 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-28667089
14.
Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane.
Biochem J
; 374(Pt 3): 793-7, 2003 Sep 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-12820897
15.
Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore.
Biochem J
; 375(Pt 3): 633-41, 2003 Nov 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-12892562
16.
Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein.
Nat Biotechnol
; 30(9): 876-82, 2012 Sep.
Artigo
em Inglês
| MEDLINE | ID: mdl-22922672
17.
An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice.
J Cell Sci
; 122(Pt 8): 1229-37, 2009 Apr 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-19339555
18.
Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs.
Chem Biol
; 16(5): 520-30, 2009 May 29.
Artigo
em Inglês
| MEDLINE | ID: mdl-19477416
19.
A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity.
J Biol Chem
; 279(40): 41664-9, 2004 Oct 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-15284228
20.
The chloride channel ClC-4 contributes to endosomal acidification and trafficking.
J Biol Chem
; 278(31): 29267-77, 2003 Aug 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-12746443