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Min, Kyung-Tai
Molecular & Cellular Neurobiology Lab(Min Lab)
Research Interests
  • Local protein synthesis, learning & memory, behavior, mitochondrial dynamics

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Preventing neurodegeneration in the Drosophila mutant bubblegum

Cited 115 times inthomson ciCited 112 times inthomson ci
Title
Preventing neurodegeneration in the Drosophila mutant bubblegum
Author
Min, Kyung-TaiBenzer, S
Keywords
X-LINKED ADRENOLEUKODYSTROPHY; CHILDHOOD ADRENOLEUKODYSTROPHY; BRAIN DEGENERATION; FATTY-ACIDS; ADRENOMYELONEUROPATHY; GENE; MICE
Issue Date
199906
Publisher
AMER ASSOC ADVANCEMENT SCIENCE
Citation
SCIENCE, v.284, no.5422, pp.1985 - 1988
Abstract
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.
URI
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DOI
http://dx.doi.org/10.1126/science.284.5422.1985
ISSN
0036-8075
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