Document Detail


Preventing neurodegeneration in the Drosophila mutant bubblegum.
MedLine Citation:
PMID:  10373116     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The Drosophila melanogaster recessive mutant bubblegum (bgm) exhibits adult neurodegeneration, with marked dilation of photoreceptor axons. The bubblegum mutant shows elevated levels of very long chain fatty acids (VLCFAs), as seen in the human disease adrenoleukodystrophy (ALD). In ALD, the excess can be lowered by dietary treatment with "Lorenzo's oil," a mixture of unsaturated fatty acids. Feeding the fly mutant one of the components, glyceryl trioleate oil, blocked the accumulation of excess VLCFAs as well as development of the pathology. Mutant flies thus provide a potential model system for studying mechanisms of neurodegenerative disease and screening drugs for treatment.
Authors:
K T Min; S Benzer
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Science (New York, N.Y.)     Volume:  284     ISSN:  0036-8075     ISO Abbreviation:  Science     Publication Date:  1999 Jun 
Date Detail:
Created Date:  1999-07-07     Completed Date:  1999-07-07     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0404511     Medline TA:  Science     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1985-8     Citation Subset:  IM    
Affiliation:
Division of Biology 156-29, California Institute of Technology, Pasadena, CA 91125, USA.
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MeSH Terms
Descriptor/Qualifier:
Adrenoleukodystrophy / diet therapy,  genetics
Amino Acid Sequence
Animals
Coenzyme A Ligases / chemistry,  genetics*
Disease Models, Animal
Drosophila Proteins*
Drosophila melanogaster / genetics*,  metabolism
Drug Combinations
Erucic Acids / administration & dosage,  pharmacology
Fatty Acids / metabolism*
Female
Genes, Insect
Genes, Recessive
Male
Molecular Sequence Data
Mutation
Nerve Degeneration*
Photoreceptor Cells, Invertebrate / ultrastructure
Repressor Proteins*
Saccharomyces cerevisiae Proteins*
Sex Characteristics
Triolein / administration & dosage,  pharmacology*
Chemical
Reg. No./Substance:
0/Drosophila Proteins; 0/Drug Combinations; 0/Erucic Acids; 0/Fatty Acids; 0/Lorenzo's oil; 0/Repressor Proteins; 0/Saccharomyces cerevisiae Proteins; 122-32-7/Triolein; EC 6.2.1.-/Coenzyme A Ligases; EC 6.2.1.3/FAA2 protein, S cerevisiae; EC 6.2.1.3/bgm protein, Drosophila; EC 6.2.1.3/long-chain-fatty-acid-CoA ligase
Comments/Corrections
Comment In:
Science. 1999 Jun 18;284(5422):1899, 1901   [PMID:  10400528 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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