Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Science 18 June 1999:
Vol. 284. no. 5422, pp. 1985 - 1988
DOI: 10.1126/science.284.5422.1985
Prev | Table of Contents | Next

Reports

Preventing Neurodegeneration in the Drosophila Mutant bubblegum

Kyung-Tai Min, Seymour Benzer *

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.

Division of Biology 156-29, California Institute of Technology, Pasadena, CA 91125, USA.
*   To whom correspondence should be addressed.

Read the Full Text


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Analyses of mental dysfunction-related ACSl4 in Drosophila reveal its requirement for Dpp/BMP production and visual wiring in the brain.
Y. Zhang, D. Chen, and Z. Wang (2009)
Hum. Mol. Genet. 18, 3894-3905
   Abstract »    Full Text »    PDF »
Mitochondrial Superoxide Radicals Differentially Affect Muscle Activity and Neural Function.
T. Godenschwege, R. Forde, C. P. Davis, A. Paul, K. Beckwith, and A. Duttaroy (2009)
Genetics 183, 175-184
   Abstract »    Full Text »    PDF »
De novo CoA biosynthesis is required to maintain DNA integrity during development of the Drosophila nervous system.
F. Bosveld, A. Rana, P. E. van der Wouden, W. Lemstra, M. Ritsema, H. H. Kampinga, and O. C. M. Sibon (2008)
Hum. Mol. Genet. 17, 2058-2069
   Abstract »    Full Text »    PDF »
Neuropathology in Drosophila Mutants With Increased Seizure Susceptibility.
T. Fergestad, L. Olson, K. P. Patel, R. Miller, M. J. Palladino, and B. Ganetzky (2008)
Genetics 178, 947-956
   Abstract »    Full Text »    PDF »
Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome.
P. A. Watkins, D. Maiguel, Z. Jia, and J. Pevsner (2007)
J. Lipid Res. 48, 2736-2750
   Abstract »    Full Text »    PDF »
Drosophila Niemann-Pick Type C-2 genes control sterol homeostasis and steroid biosynthesis: a model of human neurodegenerative disease.
X. Huang, J. T. Warren, J. Buchanan, L. I. Gilbert, and M. P. Scott (2007)
Development 134, 3733-3742
   Abstract »    Full Text »    PDF »
Multiple mechanisms limit the duration of wakefulness in Drosophila brain.
J. E. Zimmerman, W. Rizzo, K. R. Shockley, D. M. Raizen, N. Naidoo, M. Mackiewicz, G. A. Churchill, and A. I. Pack (2006)
Physiol Genomics 27, 337-350
   Abstract »    Full Text »    PDF »
Inaugural Article: wasted away, a Drosophila mutation in triosephosphate isomerase, causes paralysis, neurodegeneration, and early death.
J. P. Gnerer, R. A. Kreber, and B. Ganetzky (2006)
PNAS 103, 14987-14993
   Abstract »    Full Text »    PDF »
Uptake and metabolism of plasma-derived erucic acid by rat brain.
M. Y. Golovko and E. J. Murphy (2006)
J. Lipid Res. 47, 1289-1297
   Abstract »    Full Text »    PDF »
The Second Member of the Human and Murine "Bubblegum" Family Is a Testis- and Brainstem-specific Acyl-CoA Synthetase.
Z. Pei, Z. Jia, and P. A. Watkins (2006)
J. Biol. Chem. 281, 6632-6641
   Abstract »    Full Text »    PDF »
Neuropathology in Drosophila Membrane Excitability Mutants.
T. Fergestad, B. Ganetzky, and M. J. Palladino (2006)
Genetics 172, 1031-1042
   Abstract »    Full Text »    PDF »
Enigma, a mitochondrial protein affecting lifespan and oxidative stress response in Drosophila.
P. Mourikis, G. D. Hurlbut, and S. Artavanis-Tsakonas (2006)
PNAS 103, 1307-1312
   Abstract »    Full Text »    PDF »
Mitochondrial Encephalomyopathy in Drosophila.
A. M. Celotto, A. C. Frank, S. W. McGrath, T. Fergestad, W. A. Van Voorhies, K. F. Buttle, C. A. Mannella, and M. J. Palladino (2006)
J. Neurosci. 26, 810-820
   Abstract »    Full Text »    PDF »
A Drosophila model of the Niemann-Pick type C lysosome storage disease: dnpc1a is required for molting and sterol homeostasis.
X. Huang, K. Suyama, J. Buchanan, A. J. Zhu, and M. P. Scott (2005)
Development 132, 5115-5124
   Abstract »    Full Text »    PDF »
Drosophila: A "Model" Model System To Study Neurodegeneration.
A. M. Celotto and M. J. Palladino (2005)
Mol. Interv. 5, 292-303
   Abstract »    Full Text »    PDF »
Decreased expression of ABCD4 and BG1 genes early in the pathogenesis of X-linked adrenoleukodystrophy.
M. Asheuer, I. Bieche, I. Laurendeau, A. Moser, B. Hainque, M. Vidaud, and P. Aubourg (2005)
Hum. Mol. Genet. 14, 1293-1303
   Abstract »    Full Text »    PDF »
Mouse Very Long-chain Acyl-CoA Synthetase 3/Fatty Acid Transport Protein 3 Catalyzes Fatty Acid Activation but Not Fatty Acid Transport in MA-10 Cells.
Z. Pei, P. Fraisl, J. Berger, Z. Jia, S. Forss-Petter, and P. A. Watkins (2004)
J. Biol. Chem. 279, 54454-54462
   Abstract »    Full Text »    PDF »
Finding New Clock Components: Past and Future.
J. S. Takahashi (2004)
J Biol Rhythms 19, 339-347
   Abstract »    PDF »
The Acyl-CoA Synthetase "Bubblegum" (Lipidosin): FURTHER CHARACTERIZATION AND ROLE IN NEURONAL FATTY ACID {beta}-OXIDATION.
Z. Pei, N. A. Oey, M. M. Zuidervaart, Z. Jia, Y. Li, S. J. Steinberg, K. D. Smith, and P. A. Watkins (2003)
J. Biol. Chem. 278, 47070-47078
   Abstract »    Full Text »    PDF »
Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein.
J. P. Taylor, A. A. Taye, C. Campbell, P. Kazemi-Esfarjani, K. H. Fischbeck, and K.-T. Min (2003)
Genes & Dev. 17, 1463-1468
   Abstract »    Full Text »    PDF »
Identification of Bioactive Molecules by Adipogenesis Profiling of Organic Compounds.
Y. Choi, Y. Kawazoe, K. Murakami, H. Misawa, and M. Uesugi (2003)
J. Biol. Chem. 278, 7320-7324
   Abstract »    Full Text »    PDF »
blue cheese Mutations Define a Novel, Conserved Gene Involved in Progressive Neural Degeneration.
K. D. Finley, P. T. Edeen, R. C. Cumming, M. D. Mardahl-Dumesnil, B. J. Taylor, M. H. Rodriguez, C. E. Hwang, M. Benedetti, and M. McKeown (2003)
J. Neurosci. 23, 1254-1264
   Abstract »    Full Text »    PDF »
Neural Dysfunction and Neurodegeneration in Drosophila Na+/K+ ATPase Alpha Subunit Mutants.
M. J. Palladino, J. E. Bower, R. Kreber, and B. Ganetzky (2003)
J. Neurosci. 23, 1276-1286
   Abstract »    Full Text »    PDF »
Temperature-Sensitive Paralytic Mutants Are Enriched For Those Causing Neurodegeneration in Drosophila.
M. J. Palladino, T. J. Hadley, and B. Ganetzky (2002)
Genetics 161, 1197-1208
   Abstract »    Full Text »    PDF »
The Drosophila Genome Sequence: Implications for Biology and Medicine.
T. B. Kornberg and a. M. A. Krasnow (2000)
Science 287, 2218-2220
   Abstract »    Full Text »
An Exploration of the Sequence of a 2.9-Mb Region of the Genome of Drosophila melanogaster: The Adh Region.
M. Ashburner, S. Misra, J. Roote, S. E. Lewis, R. Blazej, T. Davis, C. Doyle, R. Galle, R. George, N. Harris, et al. (1999)
Genetics 153, 179-219
   Abstract »    Full Text »
Very Long-chain Acyl-CoA Synthetases. HUMAN "BUBBLEGUM" REPRESENTS A NEW FAMILY OF PROTEINS CAPABLE OF ACTIVATING VERY LONG-CHAIN FATTY ACIDS.
S. J. Steinberg, J. Morgenthaler, A. K. Heinzer, K. D. Smith, and P. A. Watkins (2000)
J. Biol. Chem. 275, 35162-35169
   Abstract »    Full Text »    PDF »
Life extension in Drosophila by feeding a drug.
H.-L. Kang, S. Benzer, and K.-T. Min (2002)
PNAS 99, 838-843
   Abstract »    Full Text »    PDF »
Age-Associated Cardiac Dysfunction in Drosophila melanogaster.
G. Paternostro, C. Vignola, D.-U. Bartsch, J. H. Omens, A. D. McCulloch, and J. C. Reed (2001)
Circ. Res. 88, 1053-1058
   Abstract »    Full Text »    PDF »


To Advertise     Find Products

Science. ISSN 0036-8075 (print), 1095-9203 (online)