Cystic Fibrosis Transmembrane Conductance Regulator
Simarna Kaur
Broschiertes Buch

Cystic Fibrosis Transmembrane Conductance Regulator

Regulation by Rab GTPases

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Cystic fibrosis (CF) is caused by the dysfunction of cystic fibrosis transmembrane conductance regulator (CFTR). The predominant mutation found in CF patients (delta F508) results in a mislocalized channel, which is unable to reach the plasma membrane. Rab proteins are small GTPases involved in vesicle transport. The regulation of CFTR by Rab GTPases-4 and 27a was evaluated in multiple cell systems: the colorectal HT-29 cells, bronchial epithelial IB3-1 and S9 cells. Over-expression of Rab proteins led to inhibition of CFTR activity by reducing its cell surface level, without affecting the tot...