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Results: 1 to 20 of 105

1.

Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3.

Suga N, Katsuno M, Koike H, Banno H, Suzuki K, Hashizume A, Mano T, Iijima M, Kawagashira Y, Hirayama M, Nakamura T, Watanabe H, Tanaka F, Sobue G.

Neuropathol Appl Neurobiol. 2014 Aug;40(5):628-39. doi: 10.1111/nan.12055.

PMID:
23617879
[PubMed - indexed for MEDLINE]
2.

Age related axonal neuropathy in spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD).

Klockgether T, Schöls L, Abele M, Bürk K, Topka H, Andres F, Amoiridis G, Lüdtke R, Riess O, Laccone F, Dichgans J.

J Neurol Neurosurg Psychiatry. 1999 Feb;66(2):222-4.

PMID:
10071104
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Frequency of SCA1, SCA2, SCA3/MJD, SCA6, SCA7, and DRPLA CAG trinucleotide repeat expansion in patients with hereditary spinocerebellar ataxia from Chinese kindreds.

Tang B, Liu C, Shen L, Dai H, Pan Q, Jing L, Ouyang S, Xia J.

Arch Neurol. 2000 Apr;57(4):540-4.

PMID:
10768629
[PubMed - indexed for MEDLINE]
4.

Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.

Rüb U, de Vos RA, Brunt ER, Sebestény T, Schöls L, Auburger G, Bohl J, Ghebremedhin E, Gierga K, Seidel K, den Dunnen W, Heinsen H, Paulson H, Deller T.

Brain Pathol. 2006 Jul;16(3):218-27.

PMID:
16911479
[PubMed - indexed for MEDLINE]
5.

Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: removal of the CAG containing exon.

Evers MM, Tran HD, Zalachoras I, Pepers BA, Meijer OC, den Dunnen JT, van Ommen GJ, Aartsma-Rus A, van Roon-Mom WM.

Neurobiol Dis. 2013 Oct;58:49-56. doi: 10.1016/j.nbd.2013.04.019. Epub 2013 May 6.

PMID:
23659897
[PubMed - indexed for MEDLINE]
Free Article
6.

Inflammatory genes are upregulated in expanded ataxin-3-expressing cell lines and spinocerebellar ataxia type 3 brains.

Evert BO, Vogt IR, Kindermann C, Ozimek L, de Vos RA, Brunt ER, Schmitt I, Klockgether T, Wüllner U.

J Neurosci. 2001 Aug 1;21(15):5389-96.

PMID:
11466410
[PubMed - indexed for MEDLINE]
Free Article
7.

Machado-Joseph disease/spinocerebellar ataxia type 3.

Paulson H.

Handb Clin Neurol. 2012;103:437-49. doi: 10.1016/B978-0-444-51892-7.00027-9. Review.

PMID:
21827905
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3.

Evert BO, Vogt IR, Vieira-Saecker AM, Ozimek L, de Vos RA, Brunt ER, Klockgether T, Wüllner U.

J Neuropathol Exp Neurol. 2003 Oct;62(10):1006-18.

PMID:
14575237
[PubMed - indexed for MEDLINE]
9.

Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).

Hübener J, Weber JJ, Richter C, Honold L, Weiss A, Murad F, Breuer P, Wüllner U, Bellstedt P, Paquet-Durand F, Takano J, Saido TC, Riess O, Nguyen HP.

Hum Mol Genet. 2013 Feb 1;22(3):508-18. doi: 10.1093/hmg/dds449. Epub 2012 Oct 24.

PMID:
23100324
[PubMed - indexed for MEDLINE]
Free Article
10.

Pattern of peripheral nerve involvement in Machado-Joseph disease: neuronopathy or distal axonopathy? A clinical and neurophysiological evaluation.

Escorcio Bezerra ML, Pedroso JL, Pinheiro DS, Braga-Neto P, Povoas Barsottini OG, Braga NI, Manzano GM.

Eur Neurol. 2013;69(3):129-33. doi: 10.1159/000345274. Epub 2012 Dec 7.

PMID:
23234876
[PubMed - indexed for MEDLINE]
11.

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.

Bichelmeier U, Schmidt T, Hübener J, Boy J, Rüttiger L, Häbig K, Poths S, Bonin M, Knipper M, Schmidt WJ, Wilbertz J, Wolburg H, Laccone F, Riess O.

J Neurosci. 2007 Jul 11;27(28):7418-28.

PMID:
17626202
[PubMed - indexed for MEDLINE]
Free Article
12.

Ataxin-7 expression analysis in controls and spinocerebellar ataxia type 7 patients.

Einum DD, Townsend JJ, Ptácek LJ, Fu YH.

Neurogenetics. 2001 Mar;3(2):83-90.

PMID:
11354830
[PubMed - indexed for MEDLINE]
13.

Axonal inclusions in spinocerebellar ataxia type 3.

Seidel K, den Dunnen WF, Schultz C, Paulson H, Frank S, de Vos RA, Brunt ER, Deller T, Kampinga HH, Rüb U.

Acta Neuropathol. 2010 Oct;120(4):449-60. doi: 10.1007/s00401-010-0717-7. Epub 2010 Jul 16.

PMID:
20635090
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.

Chai Y, Koppenhafer SL, Shoesmith SJ, Perez MK, Paulson HL.

Hum Mol Genet. 1999 Apr;8(4):673-82.

PMID:
10072437
[PubMed - indexed for MEDLINE]
Free Article
15.

Mutant ataxin-3 with an abnormally expanded polyglutamine chain disrupts dendritic development and metabotropic glutamate receptor signaling in mouse cerebellar Purkinje cells.

Konno A, Shuvaev AN, Miyake N, Miyake K, Iizuka A, Matsuura S, Huda F, Nakamura K, Yanagi S, Shimada T, Hirai H.

Cerebellum. 2014 Feb;13(1):29-41. doi: 10.1007/s12311-013-0516-5.

PMID:
23955261
[PubMed - indexed for MEDLINE]
16.

An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.

Schmidt T, Landwehrmeyer GB, Schmitt I, Trottier Y, Auburger G, Laccone F, Klockgether T, Völpel M, Epplen JT, Schöls L, Riess O.

Brain Pathol. 1998 Oct;8(4):669-79.

PMID:
9804376
[PubMed - indexed for MEDLINE]
17.

Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice.

Nguyen HP, Hübener J, Weber JJ, Grueninger S, Riess O, Weiss A.

PLoS One. 2013 Apr 23;8(4):e62043. doi: 10.1371/journal.pone.0062043. Print 2013.

PMID:
23626768
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Pathology and pathogenesis of sensory neuropathy in Friedreich's ataxia.

Morral JA, Davis AN, Qian J, Gelman BB, Koeppen AH.

Acta Neuropathol. 2010 Jul;120(1):97-108. doi: 10.1007/s00401-010-0675-0. Epub 2010 Mar 26.

PMID:
20339857
[PubMed - indexed for MEDLINE]
19.

H1152 promotes the degradation of polyglutamine-expanded ataxin-3 or ataxin-7 independently of its ROCK-inhibiting effect and ameliorates mutant ataxin-3-induced neurodegeneration in the SCA3 transgenic mouse.

Wang HL, Hu SH, Chou AH, Wang SS, Weng YH, Yeh TH.

Neuropharmacology. 2013 Jul;70:1-11. doi: 10.1016/j.neuropharm.2013.01.006. Epub 2013 Jan 21.

PMID:
23347954
[PubMed - indexed for MEDLINE]
20.

CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration.

Liman J, Deeg S, Voigt A, Voßfeldt H, Dohm CP, Karch A, Weishaupt J, Schulz JB, Bähr M, Kermer P.

J Neurochem. 2014 Jun;129(6):1013-23. doi: 10.1111/jnc.12684. Epub 2014 Mar 24.

PMID:
24548080
[PubMed - indexed for MEDLINE]
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