Chaperoning brain degeneration.
Drosophila has emerged as a première model system for the study of human neurodegenerative disease. Genes associated with neurodegeneration can be expressed in flies, causing phenotypes remarkably similar to those of the counterpart human diseases. Because human neurodegenerative diseases, including Huntington's and Parkinson's diseases, are disorders for which few cures or treatments are available, Drosophila brings to bear powerful genetics to the problem of these diseases. The molecular chaperones were the first modifiers defined that interfere in the progression of such disease phenotypes in Drosophila. Hsp70 is a potent suppressor of both polyglutamine disease and Parkinson's disease in Drosophila. These studies provide the promise of treatments for human neurodegeneration through the up-regulation of stress and chaperone pathways.
Bonini
Nancy M
NM
Department of Biology, University of Pennsylvania, Howard Hughes Medical Institute, Philadelphia 19104-6018, USA. nbonini@sas.upenn.edu
D000818:Animals; D001921:Brain / Q000473:pathology*; D004195:Disease Models, Animal; D004330:Drosophila; D018840:HSP70 Heat-Shock Proteins / Q000502:physiology*; D006801:Humans; D009419:Nerve Tissue Proteins / Q000633:toxicity; D019636:Neurodegenerative Diseases / Q000473:pathology*; D010455:Peptides / Q000502:physiology; D051843:Synucleins
D018840:HSP70 Heat-Shock Proteins; D009419:Nerve Tissue Proteins; D010455:Peptides; D051843:Synucleins; C097188:polyglutamine