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PMID: 12149445 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Review

Chaperoning brain degeneration.

Proceedings of the National Academy of Sciences of the United States of America ·Vol. 99 Suppl 4 ·2002-12-10 ·Pages 16407-11

Bonini NM

Abstract

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.

MeSH Terms
Animals Brain/pathology Disease Models, Animal Drosophila HSP70 Heat-Shock Proteins/physiology Humans Nerve Tissue Proteins/toxicity Neurodegenerative Diseases/pathology Peptides/physiology Synucleins
Chemicals
HSP70 Heat-Shock Proteins Nerve Tissue Proteins Peptides Synucleins polyglutamine
Authors & Affiliations
1 authors, click to expand affiliations / ORCID
Bonini Nancy M
Department of Biology, University of Pennsylvania, Howard Hughes Medical Institute, Philadelphia 19104-6018, USA. [email protected]
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Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
DOI
10.1073/pnas.152330499
Email
[email protected]
Published
2002-12-10
Epub
2002-00-29
Pages
16407-11
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC139901
Subset
IM
External Links
PubMed source DOI: 10.1073/pnas.152330499 PMC Full Text View journal details
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