Intranuclear aggregation of nonexpanded ataxin-3 in Marinesco bodies of the nonhuman primate substantia nigra

Experimental Neurology

Volumn 176, Issue 1, 2002, Pages 117-121

  Kettner, Mattias ^a   Willwohl, Dirk ^a   Hubbard, Gene B ^b   Rub U ^a   Dick Jr , Edward J ^c   Cox, Ann B ^d   Trottier, Yvon ^e   Auburger, Georg ^a   Braak, Heiko ^a   Schultz, Christian ^a  

^a GOETHE UNIVERSITY   (Germany)

^b SOUTHWEST NATIONAL PRIMATE RESEARCH CENTER   (United States)

^c WILFORD HALL MEDICAL CENTER   (United States)

^d AIR FORCE RESEARCH LABORATORY   (United States)

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

Author keywords

Ataxin 3; Intranuclear inclusion; Marinesco bodies; Nonhuman primates; Polyglutamine disease; Spinocerebellar ataxia; Ubiquitin

Indexed keywords

MONOCLONAL ANTIBODY; POLYGLUTAMINE; PROTEASOME; PROTEIN; PROTEIN ATAXIN 3; UBIQUITIN; UNCLASSIFIED DRUG;

AGE; ANIMAL TISSUE; ARTICLE; BABOON; CELL INCLUSION; CELL NUCLEUS; CONTROLLED STUDY; IMMUNOHISTOCHEMISTRY; IMMUNOREACTIVITY; MACACA; MACHADO JOSEPH DISEASE; MARINESCO BODY; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN ANALYSIS; PROTEIN DEGRADATION; PROTEIN DETERMINATION; PROTEIN LOCALIZATION; RHESUS MONKEY; SPINOCEREBELLAR DEGENERATION; SUBSTANTIA NIGRA; WILD TYPE;

EID: 0036318682     PISSN: 00144886     EISSN: None     Source Type: Journal    
DOI: 10.1006/exnr.2002.7916     Document Type: Article

References (22)

1. Ubiquitin, cellular inclusions and their role in neurode-generation
2. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
3. Ubiquitin immunoreactive structures in normal human brains. Distribution and developmental aspects
4. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
5. Cell death in polyglutamine diseases
6. Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains
7. Preferential recruitment of ataxin-3 independent of expanded polyglutamine: An immunohistochemical study on Marinesco Bodies
8. CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1
9. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
10. The molecular biology of the autosomal-dominant cerebellar ataxias
11. Ataxin 1 and ataxin 3 in neuronal intranuclear inclusion disease
12. Intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies in myotonic dystrophy: A quantitative morphological study
13. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
14. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
15. Filamentous tau pathology in nerve cells, astrocytes, and oligodendrocytes of aged baboons
16. Age-related progression of tau pathology in brains of baboons
17. The effect of aging on pars compacta of the substantia nigra in rhesus monkey
18. Recruitment of nonexpanded polyglutamine proteins to intranuclear aggregates in neuronal intranuclear hyaline inclusion disease
19. Increased caspase 3 and Bax immunoreactivity accompany nuclear GAPDH translocation and neuronal apoptosis in Parkinson's disease
20. Heterogeneous intra-cellular localization and expression of ataxin-3
21. Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias
22. The morphology of Marinesco bodies (paranucleolar corpuscles) in the melanin-pigmented nuclei of the brain-stem