Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism

Methods Mol Biol. 2003:232:27-37. doi: 10.1385/1-59259-394-1:27.

Authors

Marina S Gelman¹, Ron R Kopito

Affiliation

¹ Department of Biological Sciences, Stanford University, CA, USA.

PMID: 12840537
DOI: 10.1385/1-59259-394-1:27

No abstract available

Publication types

Review

MeSH terms

Cysteine Endopeptidases / metabolism
Cystic Fibrosis / genetics
Cystic Fibrosis / metabolism*
Cystic Fibrosis / therapy
Cystic Fibrosis Transmembrane Conductance Regulator / genetics
Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
Humans
Multienzyme Complexes / metabolism
Mutation
Proteasome Endopeptidase Complex
Protein Folding*
Ubiquitin / metabolism

Substances

CFTR protein, human
Multienzyme Complexes
Ubiquitin
cystic fibrosis transmembrane conductance regulator delta F508
Cystic Fibrosis Transmembrane Conductance Regulator
Cysteine Endopeptidases
Proteasome Endopeptidase Complex