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.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, Non-P.H.S.
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adrenoleukodystrophy / diet therapy
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Adrenoleukodystrophy / genetics
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Amino Acid Sequence
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Animals
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Coenzyme A Ligases / chemistry
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Coenzyme A Ligases / genetics*
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Disease Models, Animal
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Drosophila Proteins*
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Drosophila melanogaster / genetics*
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Drosophila melanogaster / metabolism
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Drug Combinations
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Erucic Acids / administration & dosage
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Erucic Acids / pharmacology
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Fatty Acids / metabolism*
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Female
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Genes, Insect
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Genes, Recessive
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Male
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Molecular Sequence Data
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Mutation
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Nerve Degeneration*
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Photoreceptor Cells, Invertebrate / ultrastructure
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Repressor Proteins*
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Saccharomyces cerevisiae Proteins*
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Sex Characteristics
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Triolein / administration & dosage
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Triolein / pharmacology*
Substances
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Drosophila Proteins
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Drug Combinations
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Erucic Acids
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Fatty Acids
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Lorenzo's oil
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Repressor Proteins
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Saccharomyces cerevisiae Proteins
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Triolein
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Coenzyme A Ligases
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FAA2 protein, S cerevisiae
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bgm protein, Drosophila
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long-chain-fatty-acid-CoA ligase