Understanding how cystic fibrosis mutations disrupt CFTR function: From single molecules to animal models

International Journal of Biochemistry and Cell Biology

Volumn 52, Issue , 2014, Pages 47-57

  Wang, Yiting ^a   Wrennall, Joe A ^a   Cai, Zhiwei ^a   Li, Hongyu ^a   Sheppard, David N ^a  

^a UNIVERSITY OF BRISTOL   (United Kingdom)

Author keywords

CFTR; CFTR knockout pigs; Chloride ion channel; Cystic fibrosis mutations; F508del CFTR

Indexed keywords

ABC TRANSPORTER; ADENOSINE TRIPHOSPHATE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; INTRINSICALLY DISORDERED PROTEIN; IVACAFTOR; PHLOXINE; PYROPHOSPHATE;

ARTICLE; BACTERIAL CLEARANCE; CHANNEL GATING; CONFORMATIONAL TRANSITION; CRYSTAL STRUCTURE; CYSTIC FIBROSIS; DIGESTIVE SYSTEM FUNCTION DISORDER; DIMERIZATION; EPITHELIUM; FAILURE TO THRIVE; FRAMESHIFT MUTATION; GENE DELETION; GENE MUTATION; HUMAN; HYDROLYSIS; INTRACELLULAR TRANSPORT; ION TRANSPORT; LUNG FUNCTION; MISSENSE MUTATION; NONSENSE MUTATION; PANCREAS FUNCTION; PATHOPHYSIOLOGY; PROTEIN PHOSPHORYLATION; PROTEIN PROCESSING; PROTEIN STABILITY; PROTEIN SYNTHESIS; ANIMAL; DISEASE MODEL; GENETICS; METABOLISM;

ANIMALIA; SUIDAE;

ANIMALS; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DISEASE MODELS, ANIMAL; HUMANS;

EID: 84901605375     PISSN: 13572725     EISSN: 18785875     Source Type: Journal    
DOI: 10.1016/j.biocel.2014.04.001     Document Type: Review

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