The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract

Human Molecular Genetics

Volumn 10, Issue 1, 2001, Pages 25-30

  Yue, Shinji ^a   Serra, Heliane G ^a   Zoghbi, Huda Y ^b   Orr, Harry T ^a  

^a UNIVERSITY OF MINNESOTA MEDICAL SCHOOL   (United States)

^b BAYLOR COLLEGE OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ATAXIN 1; GENE PRODUCT; NUCLEAR PROTEIN; POLYGLUTAMINE; RNA; UNCLASSIFIED DRUG;

ALLELE; ARTICLE; ASSAY; AUTOSOMAL DOMINANT DISORDER; CELL NUCLEUS; CONTROLLED STUDY; DEGENERATIVE DISEASE; IN VITRO STUDY; MUTANT; NERVE CELL; NONHUMAN; PRIORITY JOURNAL; PROTEIN TRANSPORT; RNA BINDING; RNA METABOLISM; SPINOCEREBELLAR DEGENERATION; TRANSGENIC MOUSE;

ATAXIA; MUS MUSCULUS;

EID: 0035168621     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/10.1.25     Document Type: Article

References (29)

1. Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
2. Evidence for a mechanism predisposing to intergenerational CAG instability in spinocerebellar ataxia type 1
3. Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation
4. SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat
5. Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals
6. Aggregation of Huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
7. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
8. Spinocerebellar ataxia type 7 (SCA7): A neurodegenerative disorder with neuronal intranuclear inclusions
9. Nuclear inclusions of the androgen receptor in spinal and bulbar muscular atrophy
10. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 CAG triplet repeat length
11. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
12. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
13. Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures
14. The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1
15. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
16. Polyglutamine expansion in ataxin-1 downregulates specific neuronal genes prior to any known pathogenic changes in spinocerebellar ataxia type 1
17. The RGG box motif of the Herpes Simplex Virus ICP27 protein mediates an RNA-binding activity and determines in vivo methylation
18. Conserved structures and diversity of functions of RNA-binding proteins
19. Heterogeneous nuclear RNA-protein fibers in chromatin-depleted nuclei
20. Association of nuclear matrix antigens with exon-containing splicing complexes
21. Proteins with leucine-rich repeats
22. A nuclear factor containing the leucine-rich repeats expressed in murine cerebellar neurons
23. Identification and characterization of an ataxin-1 interacting protein: AlUp a ubiquitin-like nuclear protein
24. A family of ubiquitin-like proteins binds the ATPase domain of Hsp70-like Stch
25. Identification of genes that modify ataxin-1-induced neurodegeneration
26. A novel protein with RNA-binding motifs interacts with ataxin-2
27. Identification and characterization of the gene causing type 1 spinocerebellar ataxia
28. Isolation of bacterial and phage proteins by homopolymer RNA-cellulose chromatography
29. Primary structure and binding activity of the hnRNP U protein: Binding RNA through RGG box