Progressive disruption of cellular protein folding in models of polyglutamine diseases

Science

Volumn 311, Issue 5766, 2006, Pages 1471-1474

  Gidalevitz, Tali ^a   Ben Zvi, Anat ^a   Ho, Kim H ^a   Brignull, Heather R ^a   Morimoto, Richard I ^a  

^a NORTHWESTERN UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

GENES; PROTEINS; QUALITY CONTROL; TOXICITY;

NEURODEGENERATIVE DISEASE; POLYGLUTAMINE PROTEINS; TEMPERATURE-SENSITIVE MUTATIONS;

DISEASES;

POLYGLUTAMINE;

PROTEIN;

ARTICLE; CAENORHABDITIS ELEGANS; GENE MUTATION; GENOME; MODEL; NERVE DEGENERATION; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN STABILITY; QUALITY CONTROL; TEMPERATURE SENSITIVE MUTANT;

ANIMALS; CAENORHABDITIS ELEGANS; CAENORHABDITIS ELEGANS PROTEINS; DISEASE MODELS, ANIMAL; DYNAMIN I; GLUTAMINE; HUMANS; MUTATION; NEURODEGENERATIVE DISEASES; PEPTIDES; PROTEIN FOLDING; TEMPERATURE; TROPOMYOSIN;

CAENORHABDITIS ELEGANS;

EID: 33644850056     PISSN: 00368075     EISSN: 10959203     Source Type: Journal    
DOI: 10.1126/science.1124514     Document Type: Article

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33. T.G. was supported by NIH Training Grant T32 HL076139 and by an Individual NRSA F32 GM075583-01; A.B.-Z. was supported by an EMBO Long-Term Fellowship, the Parkinson Foundation, and the Hereditary Disease Foundation; and H.R.B. was supported by National Institute of General Medical Sciences (NIGMS) Molecular Biology of Disease Training Grant T32 GM08061. R.I.M. was supported by grants from the NIH (NIGMS, National Institute of Neurological Disorders and Stroke, and National Institute on Aging), the Huntington Disease Society of America Coalition for the Cure, the ALS Association, and the Daniel F. and Ada L. Rice Foundation. Some nematode strains used in this work were provided by the Caenorhabditis Genetics Center, which is funded by the NIH National Center for Research Resources (NCRR). We thank J. West and members of the Morimoto laboratory for their discussion and comments on the manuscript and F. Moore for reagents.