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246 related items for PubMed ID: 22375012

  • 1. Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer.
    Sapp E, Valencia A, Li X, Aronin N, Kegel KB, Vonsattel JP, Young AB, Wexler N, DiFiglia M.
    J Biol Chem; 2012 Apr 13; 287(16):13487-99. PubMed ID: 22375012
    [Abstract] [Full Text] [Related]

  • 2. 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 13; 82(1):84-92. PubMed ID: 12091468
    [Abstract] [Full Text] [Related]

  • 3. 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 14; 5():28. PubMed ID: 22892315
    [Abstract] [Full Text] [Related]

  • 4. Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.
    Mende-Mueller LM, Toneff T, Hwang SR, Chesselet MF, Hook VY.
    J Neurosci; 2001 Mar 15; 21(6):1830-7. PubMed ID: 11245667
    [Abstract] [Full Text] [Related]

  • 5. Multiple phenotypes in Huntington disease mouse neural stem cells.
    Ritch JJ, Valencia A, Alexander J, Sapp E, Gatune L, Sangrey GR, Sinha S, Scherber CM, Zeitlin S, Sadri-Vakili G, Irimia D, Difiglia M, Kegel KB.
    Mol Cell Neurosci; 2012 May 15; 50(1):70-81. PubMed ID: 22508027
    [Abstract] [Full Text] [Related]

  • 6. Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo.
    Legleiter J, Mitchell E, Lotz GP, Sapp E, Ng C, DiFiglia M, Thompson LM, Muchowski PJ.
    J Biol Chem; 2010 May 07; 285(19):14777-90. PubMed ID: 20220138
    [Abstract] [Full Text] [Related]

  • 7. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.
    Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB, Qin ZH, Aronin N, DiFiglia M.
    Proc Natl Acad Sci U S A; 2001 Oct 23; 98(22):12784-9. PubMed ID: 11675509
    [Abstract] [Full Text] [Related]

  • 8. 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 23; 31(1):80-8. PubMed ID: 18502655
    [Abstract] [Full Text] [Related]

  • 9. 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 15; 22(18):7862-72. PubMed ID: 12223539
    [Abstract] [Full Text] [Related]

  • 10. 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. PubMed ID: 18337408
    [Abstract] [Full Text] [Related]

  • 11. Mutant huntingtin forms in vivo complexes with distinct context-dependent conformations of the polyglutamine segment.
    Persichetti F, Trettel F, Huang CC, Fraefel C, Timmers HT, Gusella JF, MacDonald ME.
    Neurobiol Dis; 1999 Oct 12; 6(5):364-75. PubMed ID: 10527804
    [Abstract] [Full Text] [Related]

  • 12. Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice.
    Zheng S, Clabough EB, Sarkar S, Futter M, Rubinsztein DC, Zeitlin SO.
    PLoS Genet; 2010 Feb 05; 6(2):e1000838. PubMed ID: 20140187
    [Abstract] [Full Text] [Related]

  • 13. Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin.
    Tanaka Y, Igarashi S, Nakamura M, Gafni J, Torcassi C, Schilling G, Crippen D, Wood JD, Sawa A, Jenkins NA, Copeland NG, Borchelt DR, Ross CA, Ellerby LM.
    Neurobiol Dis; 2006 Feb 05; 21(2):381-91. PubMed ID: 16150600
    [Abstract] [Full Text] [Related]

  • 14. Clearance of mutant huntingtin.
    Li XJ, Li H, Li S.
    Autophagy; 2010 Jul 05; 6(5):663-4. PubMed ID: 20519964
    [Abstract] [Full Text] [Related]

  • 15. Inducing huntingtin inclusion formation in primary neuronal cell culture and in vivo by high-capacity adenoviral vectors expressing truncated and full-length huntingtin with polyglutamine expansion.
    Huang B, Schiefer J, Sass C, Kosinski CM, Kochanek S.
    J Gene Med; 2008 Mar 05; 10(3):269-79. PubMed ID: 18067195
    [Abstract] [Full Text] [Related]

  • 16. Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity.
    Luo S, Vacher C, Davies JE, Rubinsztein DC.
    J Cell Biol; 2005 May 23; 169(4):647-56. PubMed ID: 15911879
    [Abstract] [Full Text] [Related]

  • 17. Calpain activation in Huntington's disease.
    Gafni J, Ellerby LM.
    J Neurosci; 2002 Jun 15; 22(12):4842-9. PubMed ID: 12077181
    [Abstract] [Full Text] [Related]

  • 18. PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD).
    Ratovitski T, Arbez N, Stewart JC, Chighladze E, Ross CA.
    Cell Cycle; 2015 Jun 15; 14(11):1716-29. PubMed ID: 25927346
    [Abstract] [Full Text] [Related]

  • 19. Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.
    Sathasivam K, Neueder A, Gipson TA, Landles C, Benjamin AC, Bondulich MK, Smith DL, Faull RL, Roos RA, Howland D, Detloff PJ, Housman DE, Bates GP.
    Proc Natl Acad Sci U S A; 2013 Feb 05; 110(6):2366-70. PubMed ID: 23341618
    [Abstract] [Full Text] [Related]

  • 20. 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 30; 32(22):7454-65. PubMed ID: 22649225
    [Abstract] [Full Text] [Related]

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