Use of protein repair therapy in the treatment of cystic fibrosis.

doi:10.1097/00008480-199806000-00005

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Article

Use of protein repair therapy in the treatment of cystic fibrosis.

Current opinion in pediatrics - 1 Jun 1998

Rubenstein R C, Zeitlin P L

Abstract excerpt

Disruption in the biosynthesis or function the cystic fibrosis transmembrane conductance regulator (CFTR) results from over 700 different mutations in the CFTR gene. It is useful to classify these mutations by the nature of the resulting defect. Understanding the molecular mechanism that leads to...

Topics

Codon, Terminator
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Gene Expression
Gentamicins
Humans
Ion Transport
Membrane Proteins
Molecular Chaperones
Mutation
Phenylbutyrates

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