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.
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