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Volumn 53, Issue 5, 2016, Pages 2944-2953

Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling

Author keywords

Calcium uptake; Mitochondria; Mutant huntingtin; Permeability transition pore; Respiration

Indexed keywords

HUNTINGTIN; CALCIUM; MUTANT PROTEIN;

EID: 84928748470     PISSN: 08937648     EISSN: 15591182     Source Type: Journal    
DOI: 10.1007/s12035-015-9188-0     Document Type: Review
Times cited : (39)

References (81)
  • 1
    • 0034094873 scopus 로고    scopus 로고
    • Glutamine repeats and neurodegeneration
    • COI: 1:CAS:528:DC%2BD3cXjs1Gmsrw%3D, PID: 10845064
    • Zoghbi HY, Orr HT (2000) Glutamine repeats and neurodegeneration. Annu Rev Neurosci 23:217–247
    • (2000) Annu Rev Neurosci , vol.23 , pp. 217-247
    • Zoghbi, H.Y.1    Orr, H.T.2
  • 3
    • 0027480960 scopus 로고
    • A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
    • MacDonald ME, Ambrose CM, Duyao MP, Myers RH, Lin C, Srinidhi L, Barnes G, Taylor SA et al (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72(6):971–983
    • (1993) Cell , vol.72 , Issue.6 , pp. 971-983
    • MacDonald, M.E.1    Ambrose, C.M.2    Duyao, M.P.3    Myers, R.H.4    Lin, C.5    Srinidhi, L.6    Barnes, G.7    Taylor, S.A.8
  • 4
    • 23844525922 scopus 로고    scopus 로고
    • Huntington's disease genetics
    • PID: 15717026
    • Myers RH (2004) Huntington's disease genetics. NeuroRx 1(2):255–262
    • (2004) NeuroRx , vol.1 , Issue.2 , pp. 255-262
    • Myers, R.H.1
  • 5
    • 77955643169 scopus 로고    scopus 로고
    • Molecular mechanisms and potential therapeutical targets in Huntington's disease
    • COI: 1:CAS:528:DC%2BC3cXhtVyku7rN, PID: 20664076
    • Zuccato C, Valenza M, Cattaneo E (2010) Molecular mechanisms and potential therapeutical targets in Huntington's disease. Physiol Rev 90(3):905–981
    • (2010) Physiol Rev , vol.90 , Issue.3 , pp. 905-981
    • Zuccato, C.1    Valenza, M.2    Cattaneo, E.3
  • 6
    • 0037408279 scopus 로고    scopus 로고
    • Transcriptional abnormalities in Huntington disease
    • COI: 1:CAS:528:DC%2BD3sXjtFeqtrs%3D, PID: 12711212
    • Sugars KL, Rubinsztein DC (2003) Transcriptional abnormalities in Huntington disease. Trends Genet 19(5):233–238
    • (2003) Trends Genet , vol.19 , Issue.5 , pp. 233-238
    • Sugars, K.L.1    Rubinsztein, D.C.2
  • 8
    • 84855395163 scopus 로고    scopus 로고
    • Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease
    • COI: 1:CAS:528:DC%2BC38XmtFCm, PID: 21997870
    • Shirendeb UP, Calkins MJ, Manczak M, Anekonda V, Dufour B, McBride JL, Mao P, Reddy PH (2012) Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease. Hum Mol Genet 21(2):406–420
    • (2012) Hum Mol Genet , vol.21 , Issue.2 , pp. 406-420
    • Shirendeb, U.P.1    Calkins, M.J.2    Manczak, M.3    Anekonda, V.4    Dufour, B.5    McBride, J.L.6    Mao, P.7    Reddy, P.H.8
  • 9
    • 78650284389 scopus 로고    scopus 로고
    • Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli
    • COI: 1:CAS:528:DC%2BC3MXosFeiug%3D%3D, PID: 21069748
    • Costa V, Giacomello M, Hudec R, Lopreiato R, Ermak G, Lim D, Malorni W, Davies KJ et al (2010) Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli. EMBO Mol Med 2(12):490–503
    • (2010) EMBO Mol Med , vol.2 , Issue.12 , pp. 490-503
    • Costa, V.1    Giacomello, M.2    Hudec, R.3    Lopreiato, R.4    Ermak, G.5    Lim, D.6    Malorni, W.7    Davies, K.J.8
  • 10
    • 79952443408 scopus 로고    scopus 로고
    • Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity
    • COI: 1:CAS:528:DC%2BC3MXit1aitLw%3D, PID: 21336284
    • Song W, Chen J, Petrilli A, Liot G, Klinglmayr E, Zhou Y, Poquiz P, Tjong J et al (2011) Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med 17(3):377–382
    • (2011) Nat Med , vol.17 , Issue.3 , pp. 377-382
    • Song, W.1    Chen, J.2    Petrilli, A.3    Liot, G.4    Klinglmayr, E.5    Zhou, Y.6    Poquiz, P.7    Tjong, J.8
  • 11
    • 79952585486 scopus 로고    scopus 로고
    • Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage
    • COI: 1:CAS:528:DC%2BC3MXjtFSguro%3D, PID: 21257639
    • Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, Reddy PH (2011) Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. Hum Mol Genet 20(7):1438–1455
    • (2011) Hum Mol Genet , vol.20 , Issue.7 , pp. 1438-1455
    • Shirendeb, U.1    Reddy, A.P.2    Manczak, M.3    Calkins, M.J.4    Mao, P.5    Tagle, D.A.6    Reddy, P.H.7
  • 12
  • 13
    • 0027477946 scopus 로고
    • Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?
    • COI: 1:CAS:528:DyaK3sXksVKgs7Y%3D, PID: 7682343
    • Beal MF, Hyman BT, Koroshetz W (1993) Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases? Trends Neurosci 16(4):125–131
    • (1993) Trends Neurosci , vol.16 , Issue.4 , pp. 125-131
    • Beal, M.F.1    Hyman, B.T.2    Koroshetz, W.3
  • 15
    • 0021883670 scopus 로고
    • Regional mitochondrial respiratory activity in Huntington's disease brain
    • COI: 1:CAS:528:DyaL2MXks12htb4%3D, PID: 2985766
    • Brennan WA Jr, Bird ED, Aprille JR (1985) Regional mitochondrial respiratory activity in Huntington's disease brain. J Neurochem 44(6):1948–1950
    • (1985) J Neurochem , vol.44 , Issue.6 , pp. 1948-1950
    • Brennan, W.A.1    Bird, E.D.2    Aprille, J.R.3
  • 16
    • 0029875381 scopus 로고    scopus 로고
    • Mitochondrial defect in Huntington's disease caudate nucleus
    • COI: 1:CAS:528:DyaK28XisFyrsbo%3D, PID: 8602759
    • Gu M, Gash MT, Mann VM, Javoy-Agid F, Cooper JM, Schapira AH (1996) Mitochondrial defect in Huntington's disease caudate nucleus. Ann Neurol 39(3):385–389
    • (1996) Ann Neurol , vol.39 , Issue.3 , pp. 385-389
    • Gu, M.1    Gash, M.T.2    Mann, V.M.3    Javoy-Agid, F.4    Cooper, J.M.5    Schapira, A.H.6
  • 17
    • 0030919567 scopus 로고    scopus 로고
    • Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia
    • COI: 1:CAS:528:DyaK2sXjsF2qtLs%3D, PID: 9153527
    • Browne SE, Bowling AC, MacGarvey U, Baik MJ, Berger SC, Muqit MM, Bird ED, Beal MF (1997) Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. Ann Neurol 41(5):646–653
    • (1997) Ann Neurol , vol.41 , Issue.5 , pp. 646-653
    • Browne, S.E.1    Bowling, A.C.2    MacGarvey, U.3    Baik, M.J.4    Berger, S.C.5    Muqit, M.M.6    Bird, E.D.7    Beal, M.F.8
  • 18
    • 31444432457 scopus 로고    scopus 로고
    • Polyglutamine expansion inhibits respiration by increasing reactive oxygen species in isolated mitochondria
    • COI: 1:CAS:528:DC%2BD28XhtFaktrs%3D, PID: 16427603
    • Puranam KL, Wu G, Strittmatter WJ, Burke JR (2006) Polyglutamine expansion inhibits respiration by increasing reactive oxygen species in isolated mitochondria. Biochem Biophys Res Commun 341(2):607–613
    • (2006) Biochem Biophys Res Commun , vol.341 , Issue.2 , pp. 607-613
    • Puranam, K.L.1    Wu, G.2    Strittmatter, W.J.3    Burke, J.R.4
  • 19
    • 33846540080 scopus 로고    scopus 로고
    • The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis
    • COI: 1:CAS:528:DC%2BD2sXotVWrsg%3D%3D, PID: 17135277
    • Rockabrand E, Slepko N, Pantalone A, Nukala VN, Kazantsev A, Marsh JL, Sullivan PG, Steffan JS et al (2007) The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. Hum Mol Genet 16(1):61–77
    • (2007) Hum Mol Genet , vol.16 , Issue.1 , pp. 61-77
    • Rockabrand, E.1    Slepko, N.2    Pantalone, A.3    Nukala, V.N.4    Kazantsev, A.5    Marsh, J.L.6    Sullivan, P.G.7    Steffan, J.S.8
  • 21
    • 36049044225 scopus 로고    scopus 로고
    • Animal models of Huntington's disease
    • COI: 1:CAS:528:DC%2BD2sXhtVSns73F, PID: 17712222
    • Ramaswamy S, McBride JL, Kordower JH (2007) Animal models of Huntington's disease. ILAR J 48(4):356–373
    • (2007) ILAR J , vol.48 , Issue.4 , pp. 356-373
    • Ramaswamy, S.1    McBride, J.L.2    Kordower, J.H.3
  • 22
    • 51349162486 scopus 로고    scopus 로고
    • Rodent genetic models of Huntington disease
    • COI: 1:CAS:528:DC%2BD1cXhtFantbrE, PID: 18638556
    • Heng MY, Detloff PJ, Albin RL (2008) Rodent genetic models of Huntington disease. Neurobiol Dis 32(1):1–9
    • (2008) Neurobiol Dis , vol.32 , Issue.1 , pp. 1-9
    • Heng, M.Y.1    Detloff, P.J.2    Albin, R.L.3
  • 23
    • 24744444740 scopus 로고    scopus 로고
    • Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin
    • COI: 1:CAS:528:DC%2BD2MXpsVCmsbk%3D, PID: 15983033
    • Milakovic T, Johnson GV (2005) Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin. J Biol Chem 280(35):30773–30782
    • (2005) J Biol Chem , vol.280 , Issue.35 , pp. 30773-30782
    • Milakovic, T.1    Johnson, G.V.2
  • 24
    • 26444441008 scopus 로고    scopus 로고
    • HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism
    • COI: 1:CAS:528:DC%2BD2MXhtVWnt7jF, PID: 16115812
    • Seong IS, Ivanova E, Lee JM, Choo YS, Fossale E, Anderson M, Gusella JF, Laramie JM et al (2005) HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum Mol Genet 14(19):2871–2880
    • (2005) Hum Mol Genet , vol.14 , Issue.19 , pp. 2871-2880
    • Seong, I.S.1    Ivanova, E.2    Lee, J.M.3    Choo, Y.S.4    Fossale, E.5    Anderson, M.6    Gusella, J.F.7    Laramie, J.M.8
  • 26
    • 84873446708 scopus 로고    scopus 로고
    • Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease
    • COI: 1:CAS:528:DC%2BC3sXhvV2qtbY%3D, PID: 23197653
    • Napoli E, Wong S, Hung C, Ross-Inta C, Bomdica P, Giulivi C (2013) Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease. Hum Mol Genet 22(5):989–1004
    • (2013) Hum Mol Genet , vol.22 , Issue.5 , pp. 989-1004
    • Napoli, E.1    Wong, S.2    Hung, C.3    Ross-Inta, C.4    Bomdica, P.5    Giulivi, C.6
  • 27
    • 0033982887 scopus 로고    scopus 로고
    • Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
    • COI: 1:STN:280:DC%2BD3c%2FpsF2ltw%3D%3D, PID: 10632104
    • Tabrizi SJ, Workman J, Hart PE, Mangiarini L, Mahal A, Bates G, Cooper JM, Schapira AH (2000) Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse. Ann Neurol 47(1):80–86
    • (2000) Ann Neurol , vol.47 , Issue.1 , pp. 80-86
    • Tabrizi, S.J.1    Workman, J.2    Hart, P.E.3    Mangiarini, L.4    Mahal, A.5    Bates, G.6    Cooper, J.M.7    Schapira, A.H.8
  • 28
    • 21544450545 scopus 로고    scopus 로고
    • p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease
    • COI: 1:CAS:528:DC%2BD2MXmvV2is78%3D, PID: 15996546
    • Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH et al (2005) p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron 47(1):29–41
    • (2005) Neuron , vol.47 , Issue.1 , pp. 29-41
    • Bae, B.I.1    Xu, H.2    Igarashi, S.3    Fujimuro, M.4    Agrawal, N.5    Taya, Y.6    Hayward, S.D.7    Moran, T.H.8
  • 29
    • 80053031331 scopus 로고    scopus 로고
    • Forebrain striatal-specific expression of mutant huntingtin protein in vivo induces cell-autonomous age-dependent alterations in sensitivity to excitotoxicity and mitochondrial function
    • PID: 21542802
    • Kim SH, Thomas CA, Andre VM, Cummings DM, Cepeda C, Levine MS, Ehrlich ME (2011) Forebrain striatal-specific expression of mutant huntingtin protein in vivo induces cell-autonomous age-dependent alterations in sensitivity to excitotoxicity and mitochondrial function. ASN Neuro 3(3):e00060
    • (2011) ASN Neuro , vol.3 , Issue.3 , pp. e00060
    • Kim, S.H.1    Thomas, C.A.2    Andre, V.M.3    Cummings, D.M.4    Cepeda, C.5    Levine, M.S.6    Ehrlich, M.E.7
  • 30
    • 84888792149 scopus 로고    scopus 로고
    • A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin
    • COI: 1:CAS:528:DC%2BC3sXhvVGqu7zL, PID: 23720495
    • Damiano M, Diguet E, Malgorn C, D'Aurelio M, Galvan L, Petit F, Benhaim L, Guillermier M et al (2013) A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin. Hum Mol Genet 22(19):3869–3882
    • (2013) Hum Mol Genet , vol.22 , Issue.19 , pp. 3869-3882
    • Damiano, M.1    Diguet, E.2    Malgorn, C.3    D'Aurelio, M.4    Galvan, L.5    Petit, F.6    Benhaim, L.7    Guillermier, M.8
  • 32
    • 3543141113 scopus 로고    scopus 로고
    • Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
    • COI: 1:CAS:528:DC%2BD2cXlt1WlsL4%3D, PID: 15163634
    • Choo YS, Johnson GV, MacDonald M, Detloff PJ, Lesort M (2004) Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet 13(14):1407–1420
    • (2004) Hum Mol Genet , vol.13 , Issue.14 , pp. 1407-1420
    • Choo, Y.S.1    Johnson, G.V.2    MacDonald, M.3    Detloff, P.J.4    Lesort, M.5
  • 33
    • 33745392939 scopus 로고    scopus 로고
    • Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin
    • COI: 1:CAS:528:DC%2BD28XltlWjsrw%3D, PID: 16452635
    • Benchoua A, Trioulier Y, Zala D, Gaillard MC, Lefort N, Dufour N, Saudou F, Elalouf JM et al (2006) Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol Biol Cell 17(4):1652–1663
    • (2006) Mol Biol Cell , vol.17 , Issue.4 , pp. 1652-1663
    • Benchoua, A.1    Trioulier, Y.2    Zala, D.3    Gaillard, M.C.4    Lefort, N.5    Dufour, N.6    Saudou, F.7    Elalouf, J.M.8
  • 35
    • 40849147435 scopus 로고    scopus 로고
    • N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking
    • COI: 1:CAS:528:DC%2BD1cXjvVagurs%3D, PID: 18337408
    • Orr AL, Li S, Wang CE, Li H, Wang J, Rong J, Xu X, Mastroberardino PG et al (2008) N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J Neurosci 28(11):2783–2792
    • (2008) J Neurosci , vol.28 , Issue.11 , pp. 2783-2792
    • Orr, A.L.1    Li, S.2    Wang, C.E.3    Li, H.4    Wang, J.5    Rong, J.6    Xu, X.7    Mastroberardino, P.G.8
  • 36
    • 84903985237 scopus 로고    scopus 로고
    • Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain
    • COI: 1:CAS:528:DC%2BC2cXhtFeitbrJ, PID: 24634145
    • Lim NK, Hung LW, Pang TY, Mclean CA, Liddell JR, Hilton JB, Li QX, White AR et al (2014) Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain. Hum Mol Genet 23(15):4051–4063
    • (2014) Hum Mol Genet , vol.23 , Issue.15 , pp. 4051-4063
    • Lim, N.K.1    Hung, L.W.2    Pang, T.Y.3    Mclean, C.A.4    Liddell, J.R.5    Hilton, J.B.6    Li, Q.X.7    White, A.R.8
  • 37
    • 51749107237 scopus 로고    scopus 로고
    • Increased glucose metabolism and ATP level in brain tissue of Huntington's disease transgenic mice
    • COI: 1:CAS:528:DC%2BD1cXht1SnsbrF, PID: 18721135
    • Olah J, Klivenyi P, Gardian G, Vecsei L, Orosz F, Kovacs GG, Westerhoff HV, Ovadi J (2008) Increased glucose metabolism and ATP level in brain tissue of Huntington's disease transgenic mice. FEBS J 275(19):4740–4755
    • (2008) FEBS J , vol.275 , Issue.19 , pp. 4740-4755
    • Olah, J.1    Klivenyi, P.2    Gardian, G.3    Vecsei, L.4    Orosz, F.5    Kovacs, G.G.6    Westerhoff, H.V.7    Ovadi, J.8
  • 38
    • 0034994207 scopus 로고    scopus 로고
    • Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production
    • COI: 1:CAS:528:DC%2BD3MXjsF2lt70%3D, PID: 11358447
    • Guidetti P, Charles V, Chen EY, Reddy PH, Kordower JH, Whetsell WO Jr, Schwarcz R, Tagle DA (2001) Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production. Exp Neurol 169(2):340–350
    • (2001) Exp Neurol , vol.169 , Issue.2 , pp. 340-350
    • Guidetti, P.1    Charles, V.2    Chen, E.Y.3    Reddy, P.H.4    Kordower, J.H.5    Whetsell, W.O.6    Schwarcz, R.7    Tagle, D.A.8
  • 39
    • 33947200596 scopus 로고    scopus 로고
    • Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice
    • COI: 1:CAS:528:DC%2BD2sXksFSqtro%3D, PID: 17394466
    • Oliveira JM, Jekabsons MB, Chen S, Lin A, Rego AC, Goncalves J, Ellerby LM, Nicholls DG (2007) Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice. J Neurochem 101(1):241–249
    • (2007) J Neurochem , vol.101 , Issue.1 , pp. 241-249
    • Oliveira, J.M.1    Jekabsons, M.B.2    Chen, S.3    Lin, A.4    Rego, A.C.5    Goncalves, J.6    Ellerby, L.M.7    Nicholls, D.G.8
  • 40
  • 41
    • 84908310743 scopus 로고    scopus 로고
    • Impaired brain energy metabolism in the BACHD mouse model of Huntington's disease: critical role of astrocyte-neuron interactions
    • COI: 1:CAS:528:DC%2BC2cXhtVagtr7N, PID: 24938402
    • Boussicault L, Herard AS, Calingasan N, Petit F, Malgorn C, Merienne N, Jan C, Gaillard MC et al (2014) Impaired brain energy metabolism in the BACHD mouse model of Huntington's disease: critical role of astrocyte-neuron interactions. J Cereb Blood Flow Metab 34(9):1500–1510
    • (2014) J Cereb Blood Flow Metab , vol.34 , Issue.9 , pp. 1500-1510
    • Boussicault, L.1    Herard, A.S.2    Calingasan, N.3    Petit, F.4    Malgorn, C.5    Merienne, N.6    Jan, C.7    Gaillard, M.C.8
  • 42
    • 33750437278 scopus 로고    scopus 로고
    • Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration
    • COI: 1:CAS:528:DC%2BD28Xht1aksrnP, PID: 17055784
    • Weydt P, Pineda VV, Torrence AE, Libby RT, Satterfield TF, Lazarowski ER, Gilbert ML, Morton GJ et al (2006) Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab 4(5):349–362
    • (2006) Cell Metab , vol.4 , Issue.5 , pp. 349-362
    • Weydt, P.1    Pineda, V.V.2    Torrence, A.E.3    Libby, R.T.4    Satterfield, T.F.5    Lazarowski, E.R.6    Gilbert, M.L.7    Morton, G.J.8
  • 48
    • 35348941757 scopus 로고    scopus 로고
    • Clinical correlates of mitochondrial function in Huntington's disease muscle
    • PID: 17557337
    • Turner C, Cooper JM, Schapira AH (2007) Clinical correlates of mitochondrial function in Huntington's disease muscle. Mov Disord 22(12):1715–1721
    • (2007) Mov Disord , vol.22 , Issue.12 , pp. 1715-1721
    • Turner, C.1    Cooper, J.M.2    Schapira, A.H.3
  • 50
    • 4444302167 scopus 로고    scopus 로고
    • Deranged neuronal calcium signaling and Huntington disease
    • COI: 1:CAS:528:DC%2BD2cXntFequ70%3D, PID: 15336977
    • Bezprozvanny I, Hayden MR (2004) Deranged neuronal calcium signaling and Huntington disease. Biochem Biophys Res Commun 322(4):1310–1317
    • (2004) Biochem Biophys Res Commun , vol.322 , Issue.4 , pp. 1310-1317
    • Bezprozvanny, I.1    Hayden, M.R.2
  • 51
    • 45049085513 scopus 로고    scopus 로고
    • Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease
    • COI: 1:CAS:528:DC%2BD1cXnsF2lt7k%3D, PID: 18502655
    • Zhang H, Li Q, Graham RK, Slow E, Hayden MR, Bezprozvanny I (2008) Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease. Neurobiol Dis 31(1):80–88
    • (2008) Neurobiol Dis , vol.31 , Issue.1 , pp. 80-88
    • Zhang, H.1    Li, Q.2    Graham, R.K.3    Slow, E.4    Hayden, M.R.5    Bezprozvanny, I.6
  • 52
    • 0041963057 scopus 로고    scopus 로고
    • Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1
    • COI: 1:CAS:528:DC%2BD3sXlvFyrs7Y%3D, PID: 12873381
    • Tang TS, Tu H, Chan EY, Maximov A, Wang Z, Wellington CL, Hayden MR, Bezprozvanny I (2003) Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron 39(2):227–239
    • (2003) Neuron , vol.39 , Issue.2 , pp. 227-239
    • Tang, T.S.1    Tu, H.2    Chan, E.Y.3    Maximov, A.4    Wang, Z.5    Wellington, C.L.6    Hayden, M.R.7    Bezprozvanny, I.8
  • 53
    • 80051946060 scopus 로고    scopus 로고
    • Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter
    • COI: 1:CAS:528:DC%2BC3MXnslSktLo%3D, PID: 21685886
    • Baughman JM, Perocchi F, Girgis HS, Plovanich M, Belcher-Timme CA, Sancak Y, Bao XR, Strittmatter L et al (2011) Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter. Nature 476(7360):341–345
    • (2011) Nature , vol.476 , Issue.7360 , pp. 341-345
    • Baughman, J.M.1    Perocchi, F.2    Girgis, H.S.3    Plovanich, M.4    Belcher-Timme, C.A.5    Sancak, Y.6    Bao, X.R.7    Strittmatter, L.8
  • 54
    • 80051936634 scopus 로고    scopus 로고
    • A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter
    • De SD, Raffaello A, Teardo E, Szabo I, Rizzuto R (2011) A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter. Nature 476(7360):336–340
    • (2011) Nature , vol.476 , Issue.7360 , pp. 336-340
    • De, S.D.1    Raffaello, A.2    Teardo, E.3    Szabo, I.4    Rizzuto, R.5
  • 55
    • 0032827410 scopus 로고    scopus 로고
    • Mitochondrial transport of cations: channels, exchangers, and permeability transition
    • COI: 1:CAS:528:DyaK1MXmvVymtLw%3D, PID: 10508231
    • Bernardi P (1999) Mitochondrial transport of cations: channels, exchangers, and permeability transition. Physiol Rev 79(4):1127–1155
    • (1999) Physiol Rev , vol.79 , Issue.4 , pp. 1127-1155
    • Bernardi, P.1
  • 56
    • 0038143180 scopus 로고    scopus 로고
    • The relationship between free and total calcium concentrations in the matrix of liver and brain mitochondria
    • COI: 1:CAS:528:DC%2BD3sXjvVSks7o%3D, PID: 12660243
    • Chalmers S, Nicholls DG (2003) The relationship between free and total calcium concentrations in the matrix of liver and brain mitochondria. J Biol Chem 278(21):19062–19070
    • (2003) J Biol Chem , vol.278 , Issue.21 , pp. 19062-19070
    • Chalmers, S.1    Nicholls, D.G.2
  • 57
    • 0026742968 scopus 로고
    • The permeability transition in heart mitochondria is regulated synergistically by ADP and cyclosporin A
    • COI: 1:CAS:528:DyaK38Xls1ems7w%3D, PID: 1644813
    • Novgorodov SA, Gudz TI, Milgrom YM, Brierley GP (1992) The permeability transition in heart mitochondria is regulated synergistically by ADP and cyclosporin A. J Biol Chem 267(23):16274–16282
    • (1992) J Biol Chem , vol.267 , Issue.23 , pp. 16274-16282
    • Novgorodov, S.A.1    Gudz, T.I.2    Milgrom, Y.M.3    Brierley, G.P.4
  • 58
    • 0037328244 scopus 로고    scopus 로고
    • In vitro effects of polyglutamine tracts on Ca2 + -dependent depolarization of rat and human mitochondria: relevance to Huntington's disease
    • COI: 1:CAS:528:DC%2BD3sXmsVeqtA%3D%3D, PID: 12559971
    • Panov AV, Burke JR, Strittmatter WJ, Greenamyre JT (2003) In vitro effects of polyglutamine tracts on Ca2 + -dependent depolarization of rat and human mitochondria: relevance to Huntington's disease. Arch Biochem Biophys 410(1):1–6
    • (2003) Arch Biochem Biophys , vol.410 , Issue.1 , pp. 1-6
    • Panov, A.V.1    Burke, J.R.2    Strittmatter, W.J.3    Greenamyre, J.T.4
  • 59
    • 33845933438 scopus 로고    scopus 로고
    • Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences
    • COI: 1:CAS:528:DC%2BD28XhtF2gtbvI, PID: 16973623
    • Milakovic T, Quintanilla RA, Johnson GV (2006) Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. J Biol Chem 281(46):34785–34795
    • (2006) J Biol Chem , vol.281 , Issue.46 , pp. 34785-34795
    • Milakovic, T.1    Quintanilla, R.A.2    Johnson, G.V.3
  • 60
    • 41949126549 scopus 로고    scopus 로고
    • Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease
    • COI: 1:CAS:528:DC%2BD1cXit1Omtbg%3D, PID: 18156184
    • Lim D, Fedrizzi L, Tartari M, Zuccato C, Cattaneo E, Brini M, Carafoli E (2008) Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease. J Biol Chem 283(9):5780–5789
    • (2008) J Biol Chem , vol.283 , Issue.9 , pp. 5780-5789
    • Lim, D.1    Fedrizzi, L.2    Tartari, M.3    Zuccato, C.4    Cattaneo, E.5    Brini, M.6    Carafoli, E.7
  • 63
    • 37249083913 scopus 로고    scopus 로고
    • Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease
    • COI: 1:CAS:528:DC%2BD1cXmt10%3D, PID: 18077673
    • Fernandes HB, Baimbridge KG, Church J, Hayden MR, Raymond LA (2007) Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease. J Neurosci 27(50):13614–13623
    • (2007) J Neurosci , vol.27 , Issue.50 , pp. 13614-13623
    • Fernandes, H.B.1    Baimbridge, K.G.2    Church, J.3    Hayden, M.R.4    Raymond, L.A.5
  • 64
    • 84889876549 scopus 로고    scopus 로고
    • Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease
    • Quintanilla RA, Jin YN, von BR, Johnson GV (2013) Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease. Mol Neurodegener 845
    • (2013) Mol Neurodegener , pp. 845
    • Quintanilla, R.A.1    Jin, Y.N.2    von, B.R.3    Johnson, G.V.4
  • 65
    • 20744433946 scopus 로고    scopus 로고
    • Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease
    • COI: 1:CAS:528:DC%2BD2MXlsFemsrs%3D, PID: 15935052
    • Brustovetsky N, LaFrance R, Purl KJ, Brustovetsky T, Keene CD, Low WC, Dubinsky JM (2005) Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease. J Neurochem 93(6):1361–1370
    • (2005) J Neurochem , vol.93 , Issue.6 , pp. 1361-1370
    • Brustovetsky, N.1    LaFrance, R.2    Purl, K.J.3    Brustovetsky, T.4    Keene, C.D.5    Low, W.C.6    Dubinsky, J.M.7
  • 66
    • 33646136884 scopus 로고    scopus 로고
    • Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons
    • COI: 1:CAS:528:DC%2BD28XktVyjs7o%3D, PID: 16473015
    • Chang DT, Rintoul GL, Pandipati S, Reynolds IJ (2006) Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol Dis 22(2):388–400
    • (2006) Neurobiol Dis , vol.22 , Issue.2 , pp. 388-400
    • Chang, D.T.1    Rintoul, G.L.2    Pandipati, S.3    Reynolds, I.J.4
  • 67
    • 84873312255 scopus 로고    scopus 로고
    • Dysregulation of mitochondrial calcium signaling and superoxide flashes cause mitochondrial genomic DNA damage in Huntington disease
    • COI: 1:CAS:528:DC%2BC3sXhvFems7s%3D, PID: 23250749
    • Wang JQ, Chen Q, Wang X, Wang QC, Wang Y, Cheng HP, Guo C, Sun Q et al (2013) Dysregulation of mitochondrial calcium signaling and superoxide flashes cause mitochondrial genomic DNA damage in Huntington disease. J Biol Chem 288(5):3070–3084
    • (2013) J Biol Chem , vol.288 , Issue.5 , pp. 3070-3084
    • Wang, J.Q.1    Chen, Q.2    Wang, X.3    Wang, Q.C.4    Wang, Y.5    Cheng, H.P.6    Guo, C.7    Sun, Q.8
  • 68
    • 0030004759 scopus 로고    scopus 로고
    • Involvement of cyclophilin D in the activation of a mitochondrial pore by Ca2+ and oxidant stress
    • COI: 1:CAS:528:DyaK28Xjt1Krtrw%3D, PID: 8665934
    • Tanveer A, Virji S, Andreeva L, Totty NF, Hsuan JJ, Ward JM, Crompton M (1996) Involvement of cyclophilin D in the activation of a mitochondrial pore by Ca2+ and oxidant stress. Eur J Biochem 238(1):166–172
    • (1996) Eur J Biochem , vol.238 , Issue.1 , pp. 166-172
    • Tanveer, A.1    Virji, S.2    Andreeva, L.3    Totty, N.F.4    Hsuan, J.J.5    Ward, J.M.6    Crompton, M.7
  • 69
    • 21244446551 scopus 로고    scopus 로고
    • Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D
    • COI: 1:CAS:528:DC%2BD2MXjvFektro%3D, PID: 15792954
    • Basso E, Fante L, Fowlkes J, Petronilli V, Forte MA, Bernardi P (2005) Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D. J Biol Chem 280(19):18558–18561
    • (2005) J Biol Chem , vol.280 , Issue.19 , pp. 18558-18561
    • Basso, E.1    Fante, L.2    Fowlkes, J.3    Petronilli, V.4    Forte, M.A.5    Bernardi, P.6
  • 72
    • 16044373842 scopus 로고    scopus 로고
    • Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
    • COI: 1:CAS:528:DyaK28XmvVOrtL0%3D, PID: 8898202
    • Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C, Lawton M, Trottier Y et al (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87(3):493–506
    • (1996) Cell , vol.87 , Issue.3 , pp. 493-506
    • Mangiarini, L.1    Sathasivam, K.2    Seller, M.3    Cozens, B.4    Harper, A.5    Hetherington, C.6    Lawton, M.7    Trottier, Y.8
  • 73
    • 0033054555 scopus 로고    scopus 로고
    • Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
    • COI: 1:CAS:528:DyaK1MXhslKnsb0%3D, PID: 9949199
    • Schilling G, Becher MW, Sharp AH, Jinnah HA, Duan K, Kotzuk JA, Slunt HH, Ratovitski T et al (1999) Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 8(3):397–407
    • (1999) Hum Mol Genet , vol.8 , Issue.3 , pp. 397-407
    • Schilling, G.1    Becher, M.W.2    Sharp, A.H.3    Jinnah, H.A.4    Duan, K.5    Kotzuk, J.A.6    Slunt, H.H.7    Ratovitski, T.8
  • 74
    • 53349129398 scopus 로고    scopus 로고
    • Neocortical expression of mutant huntingtin is not required for alterations in striatal gene expression or motor dysfunction in a transgenic mouse
    • COI: 1:CAS:528:DC%2BD1cXht1SitrvK, PID: 18632688
    • Brown TB, Bogush AI, Ehrlich ME (2008) Neocortical expression of mutant huntingtin is not required for alterations in striatal gene expression or motor dysfunction in a transgenic mouse. Hum Mol Genet 17(20):3095–3104
    • (2008) Hum Mol Genet , vol.17 , Issue.20 , pp. 3095-3104
    • Brown, T.B.1    Bogush, A.I.2    Ehrlich, M.E.3
  • 75
    • 0033136692 scopus 로고    scopus 로고
    • A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
    • COI: 1:CAS:528:DyaK1MXjvVSjurw%3D, PID: 10402204
    • Hodgson JG, Agopyan N, Gutekunst CA, Leavitt BR, LePiane F, Singaraja R, Smith DJ, Bissada N et al (1999) A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23(1):181–192
    • (1999) Neuron , vol.23 , Issue.1 , pp. 181-192
    • Hodgson, J.G.1    Agopyan, N.2    Gutekunst, C.A.3    Leavitt, B.R.4    LePiane, F.5    Singaraja, R.6    Smith, D.J.7    Bissada, N.8
  • 76
    • 10744227174 scopus 로고    scopus 로고
    • Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
    • COI: 1:CAS:528:DC%2BD3sXkvVSmtr0%3D, PID: 12812983
    • Slow EJ, van Raamsdonk J, Rogers D, Coleman SH, Graham RK, Deng Y, Oh R, Bissada N et al (2003) Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet 12(13):1555–1567
    • (2003) Hum Mol Genet , vol.12 , Issue.13 , pp. 1555-1567
    • Slow, E.J.1    van Raamsdonk, J.2    Rogers, D.3    Coleman, S.H.4    Graham, R.K.5    Deng, Y.6    Oh, R.7    Bissada, N.8
  • 77
    • 46749157501 scopus 로고    scopus 로고
    • Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice
    • COI: 1:CAS:528:DC%2BD1cXns1ygu7Y%3D, PID: 18550760
    • Gray M, Shirasaki DI, Cepeda C, Andre VM, Wilburn B, Lu XH, Tao J, Yamazaki I et al (2008) Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J Neurosci 28(24):6182–6195
    • (2008) J Neurosci , vol.28 , Issue.24 , pp. 6182-6195
    • Gray, M.1    Shirasaki, D.I.2    Cepeda, C.3    Andre, V.M.4    Wilburn, B.5    Lu, X.H.6    Tao, J.7    Yamazaki, I.8
  • 78
    • 0032938295 scopus 로고    scopus 로고
    • Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
    • COI: 1:CAS:528:DyaK1MXoslCmsA%3D%3D, PID: 9887339
    • Wheeler VC, Auerbach W, White JK, Srinidhi J, Auerbach A, Ryan A, Duyao MP, Vrbanac V et al (1999) Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet 8(1):115–122
    • (1999) Hum Mol Genet , vol.8 , Issue.1 , pp. 115-122
    • Wheeler, V.C.1    Auerbach, W.2    White, J.K.3    Srinidhi, J.4    Auerbach, A.5    Ryan, A.6    Duyao, M.P.7    Vrbanac, V.8
  • 80
    • 84868110606 scopus 로고    scopus 로고
    • A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease
    • COI: 1:CAS:528:DC%2BC38Xhs1CjurnO, PID: 23115180
    • Yu-Taeger L, Petrasch-Parwez E, Osmand AP, Redensek A, Metzger S, Clemens LE, Park L, Howland D et al (2012) A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease. J Neurosci 32(44):15426–15438
    • (2012) J Neurosci , vol.32 , Issue.44 , pp. 15426-15438
    • Yu-Taeger, L.1    Petrasch-Parwez, E.2    Osmand, A.P.3    Redensek, A.4    Metzger, S.5    Clemens, L.E.6    Park, L.7    Howland, D.8


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