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Items: 1 to 20 of 246

1.

Expansion of the polyQ repeat in ataxin-2 alters its Golgi localization, disrupts the Golgi complex and causes cell death.

Huynh DP, Yang HT, Vakharia H, Nguyen D, Pulst SM.

Hum Mol Genet. 2003 Jul 1;12(13):1485-96.

PMID:
12812977
2.
3.

Adenovirus-mediated expression of mutant DRPLA proteins with expanded polyglutamine stretches in neuronally differentiated PC12 cells. Preferential intranuclear aggregate formation and apoptosis.

Sato A, Shimohata T, Koide R, Takano H, Sato T, Oyake M, Igarashi S, Tanaka K, Inuzuka T, Nawa H, Tsuji S.

Hum Mol Genet. 1999 Jun;8(6):997-1006.

PMID:
10332031
4.

Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat.

Ng H, Pulst SM, Huynh DP.

Exp Neurol. 2007 Dec;208(2):207-15. Epub 2007 Aug 28.

5.

Progress in pathogenesis studies of spinocerebellar ataxia type 1.

Cummings CJ, Orr HT, Zoghbi HY.

Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29;354(1386):1079-81. Review.

6.

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats.

Nishitoh H, Matsuzawa A, Tobiume K, Saegusa K, Takeda K, Inoue K, Hori S, Kakizuka A, Ichijo H.

Genes Dev. 2002 Jun 1;16(11):1345-55.

7.
8.

Expression of extended polyglutamine sequentially activates initiator and effector caspases.

Miyashita T, Matsui J, Ohtsuka Y, Mami U, Fujishima S, Okamura-Oho Y, Inoue T, Yamada M.

Biochem Biophys Res Commun. 1999 Apr 21;257(3):724-30.

PMID:
10208851
9.

Polyglutamine-expanded ataxin-7 activates mitochondrial apoptotic pathway of cerebellar neurons by upregulating Bax and downregulating Bcl-x(L).

Wang HL, Yeh TH, Chou AH, Kuo YL, Luo LJ, He CY, Huang PC, Li AH.

Cell Signal. 2006 Apr;18(4):541-52. Epub 2005 Jun 16.

PMID:
15964171
10.

Spinocerebellar ataxia type 2: polyQ repeat variation in the CACNA1A calcium channel modifies age of onset.

Pulst SM, Santos N, Wang D, Yang H, Huynh D, Velazquez L, Figueroa KP.

Brain. 2005 Oct;128(Pt 10):2297-303. Epub 2005 Jul 6.

PMID:
16000334
11.
12.

Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice.

Helmlinger D, Bonnet J, Mandel JL, Trottier Y, Devys D.

J Biol Chem. 2004 Dec 31;279(53):55969-77. Epub 2004 Oct 19.

13.

Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity.

Nucifora FC Jr, Ellerby LM, Wellington CL, Wood JD, Herring WJ, Sawa A, Hayden MR, Dawson VL, Dawson TM, Ross CA.

J Biol Chem. 2003 Apr 11;278(15):13047-55. Epub 2002 Dec 2.

14.

Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study.

Koyano S, Uchihara T, Fujigasaki H, Nakamura A, Yagishita S, Iwabuchi K.

Neurosci Lett. 1999 Oct 1;273(2):117-20.

PMID:
10505630
15.

Atrophic macular degeneration mutations in ELOVL4 result in the intracellular misrouting of the protein.

Ambasudhan R, Wang X, Jablonski MM, Thompson DA, Lagali PS, Wong PW, Sieving PA, Ayyagari R.

Genomics. 2004 Apr;83(4):615-25.

PMID:
15028284
16.
17.

Oxidative stimuli affect polyglutamine aggregation and cell death in human mutant ataxin-1-expressing cells.

Kim SJ, Kim TS, Hong S, Rhim H, Kim IY, Kang S.

Neurosci Lett. 2003 Sep 4;348(1):21-4.

PMID:
12893416
18.

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, La Spada AR.

J Neurosci. 2002 Jun 15;22(12):4897-905.

19.

Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis.

Farg MA, Soo KY, Warraich ST, Sundaramoorthy V, Blair IP, Atkin JD.

Hum Mol Genet. 2013 Feb 15;22(4):717-28. doi: 10.1093/hmg/dds479. Epub 2012 Nov 19.

PMID:
23172909
20.

Relationship between ataxin-1 nuclear inclusions and Purkinje cell specific proteins in SCA-1 transgenic mice.

Vig PJ, Subramony SH, Qin Z, McDaniel DO, Fratkin JD.

J Neurol Sci. 2000 Mar 15;174(2):100-10.

PMID:
10727695

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