Roles for molecular chaperones in cystic fibrosis [electronic resource] / Doug Cyr.

By: Cyr, Douglas M [spk]Material type: FilmFilmSeries: Henry Stewart talksBiomedical & life sciences collection. Molecular chaperones : principles and diseases: Publisher: London : Henry Stewart Talks, 2007Description: 1 online resource (1 streaming video file (53 min.) : color, sound)Subject(s): Molecular ChaperonesOnline resources: Click here to access online | Series
Contents:
Contents: Cystic fibrosis is a fatal homozygous recessive disorder -- Caused primarily by misfolding of mutant forms of cystic fibrosis transmembrane conductance regulator (CFTR) -- The folding defects in mutant CFTR are detected by protein quality control machines in the endoplasmic reticulum that target mutant CFTR for premature degradation -- Small molecules that are being developed as drugs to treat cystic fibrosis enable mutant CFTR to avoid recognition by the endoplasmic reticulum quality control system and function at the cell surface.
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Animated audio-visual presentation with synchronized narration.

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Contents: Cystic fibrosis is a fatal homozygous recessive disorder -- Caused primarily by misfolding of mutant forms of cystic fibrosis transmembrane conductance regulator (CFTR) -- The folding defects in mutant CFTR are detected by protein quality control machines in the endoplasmic reticulum that target mutant CFTR for premature degradation -- Small molecules that are being developed as drugs to treat cystic fibrosis enable mutant CFTR to avoid recognition by the endoplasmic reticulum quality control system and function at the cell surface.

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