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Article

Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis.

The Journal of biological chemistry - 21 Apr 2006

Zhang Hui, Schmidt Bela Z, Sun Fei, Condliffe Steven B, Butterworth Michael B, Youker Robert T, Brodsky Jeffrey L, Aridor Meir, Frizzell Raymond A

Abstract excerpt

We examined the role of the cysteine string protein (Csp) in cystic fibrosis transmembrane conductance regulator (CFTR) biogenesis in relation to another J-domain protein, Hdj-2, a recognized CFTR cochaperone. Increased expression of Csp produced a dose-dependent reduction in mature (band C) CFTR and an increase in immature (band B) CFTR. Exogenous expression of Hdj-2 also increased CFTR band B, but unlike Csp,...

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PMID: 16469739
DOI: 10.1074/jbc.M512013200

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CFTR

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