Drosophila as a model to study human brain degenerative diseases

Parkinsonism and Related Disorders

Volumn 7, Issue 3, 2001, Pages 165-169

  Min, Kyung Tai ^a  

^a NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE   (United States)

Author keywords

Adrenoleukodystrophy; Bubblegum; Creutzfeldt Jakob disease; Eggroll; Spongecake; Tay Sachs disease

Indexed keywords

LONG CHAIN FATTY ACID COENZYME A LIGASE; MESYLIC ACID ETHYL ESTER;

ADRENOLEUKODYSTROPHY; ANIMAL MODEL; BRAIN DEGENERATION; BRAIN INJURY; BRAIN REGION; CHROMOSOME 2; CONFERENCE PAPER; CREUTZFELDT JAKOB DISEASE; DATA BASE; DROSOPHILA; ELECTRON MICROSCOPY; GENE INSERTION; LIFESPAN; MICROSCOPY; MUTATION; NEUROPATHOLOGY; NIEMANN PICK DISEASE; NONHUMAN; OPTIC LOBE; PHENOTYPE; PRIORITY JOURNAL; SEQUENCE ANALYSIS; SEQUENCE HOMOLOGY; TAY SACHS DISEASE; X CHROMOSOME;

EID: 0035034362     PISSN: 13538020     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1353-8020(00)00053-5     Document Type: Conference Paper

References (38)

1. Pathology of the transmissible spongeform encephalopathies with special emphasis on ultrastructure
2. Alzheimer-type neuropathology in transgenic mice overexpressing V717F b-amyloid precursor protein
3. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation
4. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homolgue
5. Mice devoid of PrP are resistant to scrapie
6. Identification and suppression of inherited neurodegeneration in Caenorhabditis elegans
7. Drosophila inherit diseases
8. Defective glia in the Drosophila brain degeneration mutant drop-dead
9. The swiss cheese mutant causes glial hyperwrapping and brain degeneration in Drosophila
10. Defective glia include neuronal apoptosis in the repo visual system of Drosophila
11. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila
12. Purification and properties of rat liver peroxisomal very-long-chain acyl-CoA synthetase
13. The Metabolic and Molecular Bases of Inherited Disease
14. Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters
15. Retroviral-mediated gene transfer corrects very-long-chain fatty acid metabolism in adrenoleukodystrophy fibroblasts
16. Fatty acid activation
17. Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy
18. Adrenoleukodystrophy: oleic acid lowers fibroblast saturated C22-26 fatty acids
19. A two-year trial of oleic and erucic acids ('Lorenzo's Oil') as treatment for adrenoleukodystrophy
20. Nervonic acid biosynthesis by erucyl-CoA elongation in normal and quaking mouse brain microsomes. Elongation of other unsaturated fatty acyl-CoAs (mono and polysaturated)
21. Ocular histopathological studies of neonatal and childhood adrenoleukodystrophy
22. Behavioral mutations of Drosophila isolated by countercurrent distribution
23. Peroxisomol disorders: genotype, phenotype, major neuropathologic lesions, and pathogenesis
24. Adrenoleukodystrophy protein-deficient mice represent abnormality of very long chain fatty acid metabolism
25. Targeted inactivation of the X-lined adrenoleukodystrophy gene in mice
26. A mouse model for X-linked adrenoleukodystrophy
27. Structure of components of the nervous system
28. Argentophilic plaques in Creutzfeldt-Jakob disease
29. Inherited metabolic disease
30. Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease
31. Synaptic pathology and glial response to neuronal injury precede the formation of senile plaques and amyloid deposits in the aging cerebral cortex
32. Structure and function of lamellar bodies, lipid-protein complexes involved in storage and secretion of cellular lipids
33. Identification and mapping of human cDNAs homologous to Drosophila mutant genes through EST database searching