Transcriptional Repression of PGC-1α by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration

Cell

Volumn 127, Issue 1, 2006, Pages 59-69

  Cui, Libin ^a   Jeong, Hyunkyung ^a   Borovecki, Fran ^a   Parkhurst, Christopher N ^b   Tanese, Naoko ^b   Krainc, Dimitri ^a  

^a HARVARD MEDICAL SCHOOL   (United States)

^b NEW YORK UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CYCLIC AMP RESPONSIVE ELEMENT BINDING PROTEIN; HUNTINGTIN; MUTANT PROTEIN; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA COACTIVATOR 1ALPHA;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CORPUS STRIATUM; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENERGY METABOLISM; GENE EXPRESSION; HUNTINGTON CHOREA; KNOCKOUT MOUSE; LENTIVIRINAE; MOTOR DYSFUNCTION; NERVE DEGENERATION; NEUROPATHOLOGY; NEUROPROTECTION; NONHUMAN; PRIORITY JOURNAL; PROMOTER REGION; TRANSCRIPTION INITIATION; TRANSCRIPTION REGULATION; TRANSGENIC MOUSE; VIRAL GENE DELIVERY SYSTEM; VIRAL GENE THERAPY;

EID: 33749042331     PISSN: 00928674     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.cell.2006.09.015     Document Type: Article

References (42)

1. Alba M.M., and Guigo R. Comparative analysis of amino acid repeats in rodents and humans. Genome Res. 14 (2004) 549-554
2. Antonini A., Leenders K.L., Spiegel R., Meier D., Vontobel P., Weigell-Weber M., Sanchez-Pernaute R., de Yebenez J.G., Boesiger P., Weindl A., and Maguire R.P. Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease. Brain 11 (1996) 2085-2095
3. Asahara H., Santoso B., Guzman E., Du K., Cole P.A., Davidson I., and Montminy M. Chromatin-dependent cooperativity between constitutive and inducible activation domains in CREB. Mol. Cell. Biol. 21 (2001) 7892-7900
4. Browne S.E., and Beal M.F. The energetics of Huntington's disease. Neurochem. Res. 29 (2004) 531-546
5. Brustovetsky N., Brustovetsky T., Purl K.J., Capano M., Crompton M., and Dubinsky J.M. Increased susceptibility of striatal mitochondria to calcium-induced permeability transition. J. Neurosci. 23 (2003) 4858-4867
6. Choo Y.S., Johnson G.V., MacDonald M., Detloff P.J., and Lesort M. Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum. Mol. Genet. 13 (2004) 1407-1420
7. Du K., Asahara H., Jhala U.S., Wagner B.L., and Montminy M. Characterization of a CREB gain-of-function mutant with constitutive transcriptional activity in vivo. Mol. Cell. Biol. 20 (2000) 4320-4327
8. Dunah A.W., Jeong H., Griffin A., Kim Y.M., Standaert D.G., Hersch S.M., Mouradian M.M., Young A.B., Tanese N., and Krainc D. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 296 (2002) 2238-2243
9. Ferrante R.J., Kowall N.W., Beal M.F., Richardson Jr. E.P., Bird E.D., and Martin J.B. Selective sparing of a class of striatal neurons in Huntington's disease. Science 230 (1985) 561-563
10. Ferrante R.J., Andreassen O.A., Jenkins B.G., Dedeoglu A., Kuemmerle S., Kubilus J.K., Kaddurah-Daouk R., Hersch S.M., and Beal M.F. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J. Neurosci. 20 (2000) 4389-4397
11. Finck B.N., and Kelly D.P. PGC-1 coactivators: inducible regulators of energy metabolism in health and disease. J. Clin. Invest. 116 (2006) 615-622
12. Furukawa T., and Tanese N. Assembly of partial TFIID complexes in mammalian cells reveals distinct activities associated with individual TATA box-binding protein-associated factors. J. Biol. Chem. 275 (2000) 29847-29856
13. Gauthier L.R., Charrin B.C., Borrell-Pages M., Dompierre J.P., Rangone H., Cordelieres F.P., De Mey J., MacDonald M.E., Lessmann V., Humbert S., and Saudou F. Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell 118 (2004) 127-138
14. Gines S., Seong I.S., Fossale E., Ivanova E., Trettel F., Gusella J.F., Wheeler V.C., Persichetti F., and MacDonald M.E. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum. Mol. Genet. 12 (2003) 497-508
15. Grafton S.T., Mazziotta J.C., Pahl J.J., St George-Hyslop P., Haines J.L., Gusella J., Hoffman J.M., Baxter L.R., and Phelps M.E. Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease. Arch. Neurol. 49 (1992) 1161-1167
16. Gunawardena S., Her L.S., Brusch R.G., Laymon R.A., Niesman I.R., Gordesky-Gold B., Sintasath L., Bonini N.M., and Goldstein L.S. Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. Neuron 40 (2003) 25-40
17. Handschin C., Rhee J., Lin J., Tarr P.T., and Spiegelman B.M. An autoregulatory loop controls peroxisome proliferator-activated receptor gamma coactivator 1alpha expression in muscle. Proc. Natl. Acad. Sci. USA 100 (2003) 7111-7116
18. Herzig S., Long F., Jhala U.S., Hedrick S., Quinn R., Bauer A., Rudolph D., Schutz G., Yoon C., Puigserver P., et al. CREB regulates hepatic gluconeogenesis through the coactivator PGC-1. Nature 413 (2001) 179-183
19. Hodges A., Strand A.D., Aragaki A.K., Kuhn A., Sengstag T., Hughes G., Elliston L.A., Hartog C., Goldstein D.R., Thu D., et al. Regional and cellular gene expression changes in human Huntington's disease brain. Hum. Mol. Genet. 15 (2006) 965-977
20. Koroshetz W.J., Jenkins B.G., Rosen B.R., and Beal M.F. Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann. Neurol. 41 (1997) 160-165
21. Kuwert T., Lange H.W., Boecker H., Titz H., Herzog H., Aulich A., Wang B.C., Nayak U., and Feinendegen L.E. Striatal glucose consumption in chorea-free subjects at risk of Huntington's disease. J. Neurol. 241 (1993) 31-36
22. Leone T.C., Lehman J.J., Finck B.N., Schaeffer P.J., Wende A.R., Boudina S., Courtois M., Wozniak D.F., Sambandam N., Bernal-Mizrachi C., et al. PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis. PLoS Biol. 3 (2005) e101
23. Lin J., Wu P., Tarr P., St-Pierre J., Zhang J., Mootha V.K., Jager S., Vianna C., Reznick R., Manieri M., et al. Defects in adaptive energy metabolism with hyperactivity in PGC-1alpha mutant mice. Cell 119 (2004) 121-135
24. Mantamadiotis T., Lemberger T., Bleckmann S.C., Kern H., Kretz O., Martin Villalba A., Tronche F., Kellendonk C., Gau D., Kapfhammer J., et al. Disruption of CREB function in brain leads to neurodegeneration. Nat. Genet. 31 (2002) 47-54
25. Menalled L.B., Sison J.D., Dragatsis I., Zeitlin S., and Chesselet M.F. Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats. J Comp Neurol. 465 (2003) 11-26
26. Panov A.V., Gutekunst C.A., Leavitt B.R., Hayden M.R., Burke J.R., Strittmatter W.J., and Greenamyre J.T. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat. Neurosci. 5 (2002) 731-736
27. Puigserver P., and Spiegelman B.M. Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator. Endocr. Rev. 24 (2003) 78-90
28. Saluja D., Vassallo M.F., and Tanese N. Distinct subdomains of human TAFII130 are required for interactions with glutamine-rich transcriptional activators. Mol. Cell. Biol. 18 (1998) 5734-5743
29. Sandoval J., Rodriguez J.L., Tur G., Serviddio G., Pereda J., Boukaba A., Sastre J., Torres L., Franco L., and Lopez-Rodas G. RNAPol-ChIP: a novel application of chromatin immunoprecipitation to the analysis of real-time gene transcription. Nucleic Acids Res. 32 (2004) 1-8
30. Saudou F., Finkbeiner S., Devys D., and Greenberg M.E. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95 (1998) 55-66
31. Shimohata T., Nakajima T., Yamada M., Uchida C., Onodera O., Naruse S., Kimura T., Koide R., Nozaki K., Sano Y., et al. Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet. 26 (2000) 29-36
32. Sena-Esteves M., Tebbets J.C., Steffens S., Crombleholme T., and Flake A.W. Optimized large-scale production of high titer lentivirus vector pseudotypes. J. Virol. Methods 122 (2004) 131-139
33. Subramanian A., Tamayo P., Mootha V.K., Mukherjee S., Ebert B.L., Gillette M.A., Paulovich A., Pomeroy S.L., Golub T.R., Lander E.S., and Mesirov J.P. Gene set enrichment analysis: a knowledge-based approach for interpreting genome-wide expression profiles. Proc. Natl. Acad. Sci. USA 102 (2005) 15545-15550
34. Sugars K.L., and Rubinsztein D.C. Transcriptional abnormalities in Huntington disease. Trends Genet. 19 (2003) 233-238
35. Szebenyi G., Morfini G.A., Babcock A., Gould M., Selkoe K., Stenoien D.L., Young M., Faber P.W., MacDonald M.E., McPhaul M.J., and Brady S.T. Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport. Neuron 40 (2003) 41-52
36. Takahashi Y., Rayman J.B., and Dynlacht B.D. Analysis of promoter binding by the E2F and pRB families in vivo: distinct E2F proteins mediate activation and repression. Genes Dev. 14 (2000) 804-816
37. Trettel F., Rigamonti D., Hilditch-Maguire P., Wheeler V.C., Sharp A.H., Persichetti F., Cattaneo E., and MacDonald M.E. Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells. Hum. Mol. Genet. 9 (2000) 2799-2809
38. Vonsattel J.P., and DiFiglia M. Huntington disease. J. Neuropathol. Exp. Neurol. 57 (1998) 369-384
39. Wheeler V.C., White J.K., Gutekunst C.A., Vrbanac V., Weaver M., Li X.J., Li S.H., Yi H., Vonsattel J.P., Gusella J.F., et al. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum. Mol. Genet. 9 (2000) 503-513
40. Zhai W., Jeong H., Cui L., Krainc D., and Tjian R. In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets. Cell 123 (2005) 1241-1253
41. Zoghbi H.Y., and Orr H.T. Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci. 23 (2000) 217-247
42. Zucker B., Luthi-Carter R., Kama J.A., Dunah A.W., Stern E.A., Fox J.H., Standaert D.G., Young A.B., and Augood S.J. Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: neuronal selectivity and potential neuroprotective role of HAP1. Hum. Mol. Genet. 14 (2005) 179-189