A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration

Neuron

Volumn 34, Issue 6, 2002, Pages 905-919

  Watase, Kei ^a,c   Weeber, Edwin J ^b   Xu, Bisong ^c   Antalffy, Barbara ^c   Yuva Paylor, Lisa ^a   Hashimoto, Kouichi ^d   Kano, Masanobu ^d   Atkinson, Richard ^a   Sun, Yaling ^a   Armstrong, Dawna L ^c   Sweatt, J David ^b   Orr, Harry T ^e   Paylor, Richard ^a,b   Zoghbi, Huda Y ^a,b,c  

^a Department of Molecular and Human Genetics ^*  (United States)

^b Division of Neuroscience   (United States)

^c BAYLOR COLLEGE OF MEDICINE   (United States)

^d KANAZAWA UNIVERSITY   (Japan)

^e UNIVERSITY OF MINNESOTA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

POLYGLUTAMINE;

AGED; ANIMAL CELL; ARTICLE; BRAIN REGION; CELL LOSS; CLINICAL FEATURE; COGNITIVE DEFECT; CONTROLLED STUDY; DEGENERATIVE DISEASE; DISEASE COURSE; GENE LOCUS; GENE REPLICATION; MOUSE; NERVE CELL; NERVE DEGENERATION; NONHUMAN; PREMATURITY; PRIORITY JOURNAL; PURKINJE CELL; SOLUBILITY; SPINOCEREBELLAR DEGENERATION; SYNAPTIC MEMBRANE; TOXICITY;

EID: 18444386197     PISSN: 08966273     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0896-6273(02)00733-X     Document Type: Article

References (39)

1. Deficient cerebellar long-term depression and impaired motor learning in mGluR1 mutant mice
2. Executive dysfunction in spinocerebellar ataxia type 1
3. SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat
4. Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors followed by a progressive cerebellar dysfunction and histological alterations
5. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
6. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
7. Over-expression of inducible HSP70 chaperone supresses neuropathology and improves motor function in SCA1 mice
8. A thin slice preparation for patch-clamp recordings from neurons of the mammalian central nervous system
9. Identification of genes that modify ataxin-1-induced neurodegeneration
10. The dorsal hippocampus is essential for context discrimination but not for contextual conditioning
11. Clinical neuropathologic and genetic studies of a large spinocerebellar ataxia type 1 (SCA1) kindred: (CAG)n expansion and early premonitory signs and symptoms
12. Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
13. Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation
14. Persistent multiple climbing fiber innervation of cerebellar Purkinje cells in mice lacking mGluR1
15. Cognitive deficits in olivopontocerebellar atrophy: Implications for the cholinergic hypothesis of Alzheimer's dementia
16. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
17. Huntington aggregates may not predict neuronal death in Huntington's disease
18. Synaptic- and agonist-induced excitatory currents of Purkinje cells in rat cerebellar slice
19. Neurological abnormalities in a knock-in mouse model of Huntington's disease
20. Expanding our understanding of polyglutamine diseases through mouse models
21. Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1
22. Hippocampal lesions cause learning deficits in inbred mice in the Morris water maze and conditioned fear task
23. Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus
24. Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation
25. CREB-binding protein sequestration by expanded polyglutamine
26. Soluble pool of Aβ amyloid as a determinant of severity of neurodegeneration in Alzheimer's disease
27. Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment
28. SCA17 a novel autosomal cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein
29. Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity
30. DBA/2 and C57BL/6 mice differ in contextual fear but not auditory fear conditioning
31. Transient activation of cyclic AMP-dependent protein kinase during hippocampal long-term potentiation
32. Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals
33. Ubiquitination of a new form of alpha-synuclein by parkin from human brain: Implications for Parkinson's disease
34. Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures
35. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
36. Molecular analysis of juvenile Huntington disease: The major influence on (CAG)n repeat length is the sex of the affected parent
37. Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivo
38. Spinocerebellar ataxia type 1
39. Glutamine repeats and neurodegeneration