A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration

Journal of Neuroscience

Volumn 24, Issue 45, 2004, Pages 10266-10279

  Goti, Daniel ^a   Katzen, Scott M ^a   Mez, Jesse ^a   Kurtis, Noam ^a   Kiluk, Jennifer ^a   Ben Haïem, Lea ^b   Jenkins, Nancy A ^c   Copeland, Neal G ^c   Kakizuka, Akira ^d   Sharp, Alan H ^e   Ross, Christopher A ^a   Mouton, Peter R ^f   Colomer, Veronica ^a  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b INSTITUT DE GÉNÉTIQUE ET DE BIOLOGIE MOLÉCULAIRE ET CELLULAIRE   (France)

^c NATIONAL CANCER INSTITUTE   (United States)

^d KYOTO UNIVERSITY   (Japan)

^e CASE WESTERN RESERVE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^f Stereology Resource Center   (United States)

Author keywords

Ataxin 3; Cleavage fragment; Machado Joseph; Mouse model; Neurotoxicity; Spinocerebellar ataxia type 3

Indexed keywords

AMINO ACID 221; AMINO ACID DERIVATIVE; MONOCLONAL ANTIBODY; NERVE PROTEIN; POLYGLUTAMINE; PROTEIN ATAXIN 3; TYROSINE 3 MONOOXYGENASE; UNCLASSIFIED DRUG;

ARTICLE; ATAXIA; BRAIN; CELL INCLUSION; CELL NUCLEUS INCLUSION BODY; CONTROLLED STUDY; CYTOTOXICITY; DEGENERATIVE DISEASE; GENE EXPRESSION; HUMAN; HUMAN TISSUE; MACHADO JOSEPH DISEASE; MALE; MOUSE; MUTANT; NEUROBLASTOMA CELL; NONHUMAN; PHENOTYPE; PRION; PRIORITY JOURNAL; STEREOLOGY; SUBSTANTIA NIGRA; TRANSGENIC MOUSE; WEIGHT REDUCTION;

ADULT; ALTERNATIVE SPLICING; AMINO ACID SEQUENCE; ANIMALS; BRAIN CHEMISTRY; CELL LINE, TUMOR; DOSE-RESPONSE RELATIONSHIP, DRUG; EXPLORATORY BEHAVIOR; FEMALE; HAND STRENGTH; HUMANS; INTRANUCLEAR INCLUSION BODIES; MACHADO-JOSEPH DISEASE; MALE; MICE; MICE, TRANSGENIC; MIDDLE AGED; MINISATELLITE REPEATS; MOLECULAR SEQUENCE DATA; MUSCULOSKELETAL EQUILIBRIUM; NERVE TISSUE PROTEINS; NEUROBLASTOMA; NEURONS; NUCLEAR PROTEINS; PEPTIDE FRAGMENTS; PHENOTYPE; REFLEX; REPRESSOR PROTEINS; SUBCELLULAR FRACTIONS; SUBSTANTIA NIGRA; TRANSCRIPTION FACTORS; TRANSFECTION;

EID: 20844462057     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/JNEUROSCI.2734-04.2004     Document Type: Article

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