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Items: 1 to 20 of 101

1.

Huntingtin Aggregates and Mitochondrial Pathology in Skeletal Muscle but not Heart of Late-Stage R6/2 Mice.

Kojer K, Hering T, Bazenet C, Weiss A, Herrmann F, Taanman JW, Orth M.

J Huntingtons Dis. 2019 Feb 19. doi: 10.3233/JHD-180324. [Epub ahead of print]

PMID:
30814364
2.

Gene expression profiling of R6/2 transgenic mice with different CAG repeat lengths reveals genes associated with disease onset and progression in Huntington's disease.

Tang B, Seredenina T, Coppola G, Kuhn A, Geschwind DH, Luthi-Carter R, Thomas EA.

Neurobiol Dis. 2011 Jun;42(3):459-67. doi: 10.1016/j.nbd.2011.02.008. Epub 2011 Feb 18.

3.

Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage.

Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, Reddy PH.

Hum Mol Genet. 2011 Apr 1;20(7):1438-55. doi: 10.1093/hmg/ddr024. Epub 2011 Jan 21.

4.

Inclusion formation in Huntington's disease R6/2 mouse muscle cultures.

Orth M, Cooper JM, Bates GP, Schapira AH.

J Neurochem. 2003 Oct;87(1):1-6.

5.

Soluble N-terminal fragment of mutant Huntingtin protein impairs mitochondrial axonal transport in cultured hippocampal neurons.

Tian J, Yan YP, Zhou R, Lou HF, Rong Y, Zhang BR.

Neurosci Bull. 2014 Feb;30(1):74-80. doi: 10.1007/s12264-013-1393-0. Epub 2013 Dec 21.

6.
7.

The dynamics of early-state transcriptional changes and aggregate formation in a Huntington's disease cell model.

van Hagen M, Piebes DGE, de Leeuw WC, Vuist IM, van Roon-Mom WMC, Moerland PD, Verschure PJ.

BMC Genomics. 2017 May 12;18(1):373. doi: 10.1186/s12864-017-3745-z.

8.

Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.

Moffitt H, McPhail GD, Woodman B, Hobbs C, Bates GP.

PLoS One. 2009 Nov 30;4(11):e8025. doi: 10.1371/journal.pone.0008025.

9.

Increased caspase-2, calpain activations and decreased mitochondrial complex II activity in cells expressing exogenous huntingtin exon 1 containing CAG repeat in the pathogenic range.

Majumder P, Raychaudhuri S, Chattopadhyay B, Bhattacharyya NP.

Cell Mol Neurobiol. 2007 Dec;27(8):1127-45. Epub 2007 Sep 28.

PMID:
17902043
10.

Mitochondrial cristae remodelling is associated with disrupted OPA1 oligomerisation in the Huntington's disease R6/2 fragment model.

Hering T, Kojer K, Birth N, Hallitsch J, Taanman JW, Orth M.

Exp Neurol. 2017 Feb;288:167-175. doi: 10.1016/j.expneurol.2016.10.017. Epub 2016 Nov 23.

PMID:
27889468
11.

Removal of the Mitochondrial Fission Factor Mff Exacerbates Neuronal Loss and Neurological Phenotypes in a Huntington's Disease Mouse Model.

Cha MY, Chen H, Chan D.

PLoS Curr. 2018 Jul 26;10. pii: ecurrents.hd.a4e15b80c4915c828d39754942c6631f. doi: 10.1371/currents.hd.a4e15b80c4915c828d39754942c6631f.

12.

Drp1/Fis1-mediated mitochondrial fragmentation leads to lysosomal dysfunction in cardiac models of Huntington's disease.

Joshi AU, Ebert AE, Haileselassie B, Mochly-Rosen D.

J Mol Cell Cardiol. 2019 Feb;127:125-133. doi: 10.1016/j.yjmcc.2018.12.004. Epub 2018 Dec 11.

PMID:
30550751
14.

Effects of overexpression of huntingtin proteins on mitochondrial integrity.

Wang H, Lim PJ, Karbowski M, Monteiro MJ.

Hum Mol Genet. 2009 Feb 15;18(4):737-52. doi: 10.1093/hmg/ddn404. Epub 2008 Nov 27.

15.

N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.

Orr AL, Li S, Wang CE, Li H, Wang J, Rong J, Xu X, Mastroberardino PG, Greenamyre JT, Li XJ.

J Neurosci. 2008 Mar 12;28(11):2783-92. doi: 10.1523/JNEUROSCI.0106-08.2008.

16.

Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease.

Rattray I, Smith EJ, Crum WR, Walker TA, Gale R, Bates GP, Modo M.

PLoS One. 2013 Dec 19;8(12):e84726. doi: 10.1371/journal.pone.0084726. eCollection 2013.

17.

Ubiquilin-1 overexpression increases the lifespan and delays accumulation of Huntingtin aggregates in the R6/2 mouse model of Huntington's disease.

Safren N, El Ayadi A, Chang L, Terrillion CE, Gould TD, Boehning DF, Monteiro MJ.

PLoS One. 2014 Jan 27;9(1):e87513. doi: 10.1371/journal.pone.0087513. eCollection 2014.

18.

Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease.

Shirendeb UP, Calkins MJ, Manczak M, Anekonda V, Dufour B, McBride JL, Mao P, Reddy PH.

Hum Mol Genet. 2012 Jan 15;21(2):406-20. doi: 10.1093/hmg/ddr475. Epub 2011 Oct 13.

19.

Essential sequence of the N-terminal cytoplasmic localization-related domain of huntingtin and its effect on huntingtin aggregates.

Yan Y, Peng D, Tian J, Chi J, Tan J, Yin X, Pu J, Xia K, Zhang B.

Sci China Life Sci. 2011 Apr;54(4):342-50. doi: 10.1007/s11427-011-4151-4. Epub 2011 Mar 8.

PMID:
21509658
20.

Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease.

Yin X, Manczak M, Reddy PH.

Hum Mol Genet. 2016 May 1;25(9):1739-53. doi: 10.1093/hmg/ddw045. Epub 2016 Feb 16.

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