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Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin.

Benchoua A, Trioulier Y, Zala D, Gaillard MC, Lefort N, Dufour N, Saudou F, Elalouf JM, Hirsch E, Hantraye P, Déglon N, Brouillet E.

Mol Biol Cell. 2006 Apr;17(4):1652-63. Epub 2006 Feb 1.


A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin.

Damiano M, Diguet E, Malgorn C, D'Aurelio M, Galvan L, Petit F, Benhaim L, Guillermier M, Houitte D, Dufour N, Hantraye P, Canals JM, Alberch J, Delzescaux T, Déglon N, Beal MF, Brouillet E.

Hum Mol Genet. 2013 Oct 1;22(19):3869-82. doi: 10.1093/hmg/ddt242. Epub 2013 May 29.


Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II.

Benchoua A, Trioulier Y, Diguet E, Malgorn C, Gaillard MC, Dufour N, Elalouf JM, Krajewski S, Hantraye P, Déglon N, Brouillet E.

Hum Mol Genet. 2008 May 15;17(10):1446-56. doi: 10.1093/hmg/ddn033. Epub 2008 Feb 11.


Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum.

Régulier E, Trottier Y, Perrin V, Aebischer P, Déglon N.

Hum Mol Genet. 2003 Nov 1;12(21):2827-36. Epub 2003 Sep 2.


Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment.

Zala D, Benchoua A, Brouillet E, Perrin V, Gaillard MC, Zurn AD, Aebischer P, Déglon N.

Neurobiol Dis. 2005 Dec;20(3):785-98. Epub 2005 Jul 11.


Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates.

Palfi S, Brouillet E, Jarraya B, Bloch J, Jan C, Shin M, Condé F, Li XJ, Aebischer P, Hantraye P, Déglon N.

Mol Ther. 2007 Aug;15(8):1444-51. Epub 2007 May 1.


Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry.

Runne H, Régulier E, Kuhn A, Zala D, Gokce O, Perrin V, Sick B, Aebischer P, Déglon N, Luthi-Carter R.

J Neurosci. 2008 Sep 24;28(39):9723-31. doi: 10.1523/JNEUROSCI.3044-08.2008.


Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin.

Milakovic T, Johnson GV.

J Biol Chem. 2005 Sep 2;280(35):30773-82. Epub 2005 Jun 27.


Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease.

Perrin V, Régulier E, Abbas-Terki T, Hassig R, Brouillet E, Aebischer P, Luthi-Carter R, Déglon N.

Mol Ther. 2007 May;15(5):903-11. Epub 2007 Mar 20.


Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease.

Zhang H, Li Q, Graham RK, Slow E, Hayden MR, Bezprozvanny I.

Neurobiol Dis. 2008 Jul;31(1):80-8. doi: 10.1016/j.nbd.2008.03.010. Epub 2008 Apr 16.


Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length.

Hansson O, Castilho RF, Korhonen L, Lindholm D, Bates GP, Brundin P.

J Neurochem. 2001 Aug;78(4):694-703.


Mitogen- and stress-activated protein kinase-1 deficiency is involved in expanded-huntingtin-induced transcriptional dysregulation and striatal death.

Roze E, Betuing S, Deyts C, Marcon E, Brami-Cherrier K, Pagès C, Humbert S, Mérienne K, Caboche J.

FASEB J. 2008 Apr;22(4):1083-93. Epub 2007 Nov 20.


Adenovirus vector-based in vitro neuronal cell model for Huntington's disease with human disease-like differential aggregation and degeneration.

Dong X, Zong S, Witting A, Lindenberg KS, Kochanek S, Huang B.

J Gene Med. 2012 Jul;14(7):468-81. doi: 10.1002/jgm.2641.


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.


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.


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.


Implication of the JNK pathway in a rat model of Huntington's disease.

Perrin V, Dufour N, Raoul C, Hassig R, Brouillet E, Aebischer P, Luthi-Carter R, Déglon N.

Exp Neurol. 2009 Jan;215(1):191-200. doi: 10.1016/j.expneurol.2008.10.008. Epub 2008 Oct 28.


Nature and cause of mitochondrial dysfunction in Huntington's disease: focusing on huntingtin and the striatum.

Oliveira JM.

J Neurochem. 2010 Jul;114(1):1-12. doi: 10.1111/j.1471-4159.2010.06741.x. Epub 2010 Apr 9. Review.

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