CFTR: Products

Cystic fibrosis transmembrane conductance regulator (CFTR) is a multi-pass transmembrane protein that functions as a chloride channel. CFTR belongs to the ATP-binding cassette (ABC) superfamily. Mutations in CFTR cause the pulmonary disease, cystic fibrosis (CF). Specifically, deletion of phenyalanine at position 508 (DeltaF508-CFTR) results in a folding defect which impairs chloride channel function. The mechanism by which channel dysfunction relates to disease symptoms is a focus of intense research. CFTR dysfunction results in disruption of ion transport and subsequent blockage of airways by secreted mucus. CFTR may also play a role in the skeletal muscle atrophy and dysfunction that characterizes CF. In addition, CFTR-mediated chloride secretion underlies fluid accumulation and cyst growth in autosomal dominant polycystic kidney disease (ADPKD).

Showing 1-7 out of 7 CFTR Products

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Human CFTR C-Terminus Antibody

IHC, IP, WBMAB2503132  
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Human CFTR R Domain Antibody

ICC/IF, IHC, IP, WBMAB166011  
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CFTRinh 172


Voltage-independent, selective CFTR chloride channel blocker
34302  
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GlyH 101


Reversible, voltage-dependent CFTR chloride channel blocker
5485 
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KM 11060


Corrects F508del-CFTR trafficking
31431  
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PG 01


CFTR potentiator; corrects F508del-CFTR conductance defect
3943 
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PPQ 102


Potent, voltage-independent CFTR chloride channel inhibitor
4303 
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