RT Journal Article
SR Electronic
T1 Preventing Neurodegeneration in the <em>Drosophila</em> Mutant <em>bubblegum</em>
JF Science
JO Science
FD American Association for the Advancement of Science
SP 1985
OP 1988
DO 10.1126/science.284.5422.1985
VO 284
IS 5422
A1 Min, Kyung-Tai
A1 Benzer, Seymour
YR 1999
UL http://science.sciencemag.org/content/284/5422/1985.abstract
AB The Drosophila melanogaster recessive mutantbubblegum (bgm) exhibits adult neurodegeneration, with marked dilation of photoreceptor axons. The bubblegummutant 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.