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2. Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation. Maglione V; Cannella M; Gradini R; Cislaghi G; Squitieri F Mech Ageing Dev; 2006 Feb; 127(2):213-6. PubMed ID: 16289252 [TBL] [Abstract][Full Text] [Related]
3. Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. Wellington CL; Ellerby LM; Gutekunst CA; Rogers D; Warby S; Graham RK; Loubser O; van Raamsdonk J; Singaraja R; Yang YZ; Gafni J; Bredesen D; Hersch SM; Leavitt BR; Roy S; Nicholson DW; Hayden MR J Neurosci; 2002 Sep; 22(18):7862-72. PubMed ID: 12223539 [TBL] [Abstract][Full Text] [Related]
4. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Hermel E; Gafni J; Propp SS; Leavitt BR; Wellington CL; Young JE; Hackam AS; Logvinova AV; Peel AL; Chen SF; Hook V; Singaraja R; Krajewski S; Goldsmith PC; Ellerby HM; Hayden MR; Bredesen DE; Ellerby LM Cell Death Differ; 2004 Apr; 11(4):424-38. PubMed ID: 14713958 [TBL] [Abstract][Full Text] [Related]
5. 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. PubMed ID: 17902043 [TBL] [Abstract][Full Text] [Related]
6. Aggregation in Huntington's disease: insights through modelling. Cajavec B; Bernard S; Herzel H Genome Inform; 2005; 16(1):262-71. PubMed ID: 16362929 [TBL] [Abstract][Full Text] [Related]
7. N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy. Liu X; Wang CE; Hong Y; Zhao T; Wang G; Gaertig MA; Sun M; Li S; Li XJ PLoS Genet; 2016 May; 12(5):e1006083. PubMed ID: 27203582 [TBL] [Abstract][Full Text] [Related]
9. Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment. Gafni J; Papanikolaou T; Degiacomo F; Holcomb J; Chen S; Menalled L; Kudwa A; Fitzpatrick J; Miller S; Ramboz S; Tuunanen PI; Lehtimäki KK; Yang XW; Park L; Kwak S; Howland D; Park H; Ellerby LM J Neurosci; 2012 May; 32(22):7454-65. PubMed ID: 22649225 [TBL] [Abstract][Full Text] [Related]
10. A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity. Bañez-Coronel M; Porta S; Kagerbauer B; Mateu-Huertas E; Pantano L; Ferrer I; Guzmán M; Estivill X; Martí E PLoS Genet; 2012; 8(2):e1002481. PubMed ID: 22383888 [TBL] [Abstract][Full Text] [Related]
11. Significantly differential diffusion of neuropathological aggregates in the brain of transgenic mice carrying N-terminal mutant huntingtin fused with green fluorescent protein. Cheng PH; Li CL; Her LS; Chang YF; Chan AW; Chen CM; Yang SH Brain Struct Funct; 2013 Jan; 218(1):283-94. PubMed ID: 22422149 [TBL] [Abstract][Full Text] [Related]
12. 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. PubMed ID: 11520890 [TBL] [Abstract][Full Text] [Related]
13. A series of N-terminal epitope tagged Hdh knock-in alleles expressing normal and mutant huntingtin: their application to understanding the effect of increasing the length of normal Huntingtin's polyglutamine stretch on CAG140 mouse model pathogenesis. Zheng S; Ghitani N; Blackburn JS; Liu JP; Zeitlin SO Mol Brain; 2012 Aug; 5():28. PubMed ID: 22892315 [TBL] [Abstract][Full Text] [Related]
14. Mechanisms for neuronal cell death and dysfunction in Huntington's disease: pathological cross-talk between the nucleus and the mitochondria? Sawa A J Mol Med (Berl); 2001 Jul; 79(7):375-81. PubMed ID: 11466559 [TBL] [Abstract][Full Text] [Related]
15. Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Jana NR; Zemskov EA; Wang Gh ; Nukina N Hum Mol Genet; 2001 May; 10(10):1049-59. PubMed ID: 11331615 [TBL] [Abstract][Full Text] [Related]
16. Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. Graham RK; Deng Y; Carroll J; Vaid K; Cowan C; Pouladi MA; Metzler M; Bissada N; Wang L; Faull RL; Gray M; Yang XW; Raymond LA; Hayden MR J Neurosci; 2010 Nov; 30(45):15019-29. PubMed ID: 21068307 [TBL] [Abstract][Full Text] [Related]
17. Comparison of huntingtin proteolytic fragments in human lymphoblast cell lines and human brain. Toneff T; Mende-Mueller L; Wu Y; Hwang SR; Bundey R; Thompson LM; Chesselet MF; Hook V J Neurochem; 2002 Jul; 82(1):84-92. PubMed ID: 12091468 [TBL] [Abstract][Full Text] [Related]
18. Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease. Reddy PH; Charles V; Williams M; Miller G; Whetsell WO; Tagle DA Philos Trans R Soc Lond B Biol Sci; 1999 Jun; 354(1386):1035-45. PubMed ID: 10434303 [TBL] [Abstract][Full Text] [Related]
19. 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. PubMed ID: 18502655 [TBL] [Abstract][Full Text] [Related]