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Article

Pharmacologic therapy for stop mutations: how much CFTR activity is enough?

27 Oct 2004

Abstract excerpt

PURPOSE OF REVIEW: The purpose of this review is to summarize the recent approaches using mutation-specific therapy to correct the genetic defect according to the molecular mechanism by which the mutation causes the defects in cystic fibrosis transmembrane conductance regulator (CFTR). Premature stop mutations (class I mutations) account for 5 to 10% of the total mutant alleles in cystic fibrosis patients, and in...

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PMID: 15510065
DOI: 10.1097/01.mcp.0000141247.22078.46

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