Article
SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis.
American journal of respiratory cell and molecular biology - 1 Mar 2006
Grubb Barbara R, Gabriel Sherif E, Mengos April, Gentzsch Martina, Randell Scott H, Van Heeckeren Anna M, Knowles Michael R, Drumm Mitchell L, Riordan John R, Boucher Richard C
Abstract excerpt
Deletion of phenylalanine 508 (deltaF508) accounts for nearly 70% of all mutations that occur in the cystic fibrosis transmembrane conductance regulator (CFTR). The deltaF508 mutation is a class II processing mutation that results in very little or no mature CFTR protein reaching the apical membrane and thus no cAMP-mediated Cl- conductance. Therapeutic strategies have been developed to enhance processing of the...
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