Publication Date:
1999-06-18
Description:
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.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Min, K T -- Benzer, S -- New York, N.Y. -- Science. 1999 Jun 18;284(5422):1985-8.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Division of Biology 156-29, California Institute of Technology, Pasadena, CA 91125, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/10373116" target="_blank"〉PubMed〈/a〉
Keywords:
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
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
