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Pregled bibliografske jedinice broj: 405337

Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration

Cui, Libin; Jeong, Hyun; Borovečki, Fran; Parkhurst, C.N.; Tanese, Naoko; Krainc, Dimitri
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration // Cell, 127 (2006), 1; 59-69 doi:10.1016/j.cell.2006.09.015 (međunarodna recenzija, članak, znanstveni)

CROSBI ID: 405337 Za ispravke kontaktirajte CROSBI podršku putem web obrasca

Naslov
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration

Autori
Cui, Libin ; Jeong, Hyun ; Borovečki, Fran ; Parkhurst, C.N. ; Tanese, Naoko ; Krainc, Dimitri

Izvornik
Cell (0092-8674) 127 (2006), 1; 59-69

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
energy metabolism; polyglutamine expansion; neurodegeneration

Sažetak
Huntington's disease (HD) is an inherited neurodegenerative disease caused by a glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered energy metabolism have been implicated in HD pathogenesis. We report here that mutant huntingtin causes disruption of mitochondrial function by inhibiting expression of PGC-1alpha, a transcriptional coactivator that regulates several metabolic processes, including mitochondrial biogenesis and respiration. Mutant huntingtin represses PGC-1alpha gene transcription by associating with the promoter and interfering with the CREB/TAF4-dependent transcriptional pathway critical for the regulation of PGC-1alpha gene expression. Crossbreeding of PGC-1alpha knockout (KO) mice with HD knockin (KI) mice leads to increased neurodegeneration of striatal neurons and motor abnormalities in the HD mice. Importantly, expression of PGC-1alpha partially reverses the toxic effects of mutant huntingtin in cultured striatal neurons. Moreover, lentiviral-mediated delivery of PGC-1alpha in the striatum provides neuroprotection in the transgenic HD mice. These studies suggest a key role for PGC-1alpha in the control of energy metabolism in the early stages of HD pathogenesis.

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti



POVEZANOST RADA

Projekti:
108-1081874-1923 - Genomska analiza transkriptoma i interaktoma u bolesnika s kompleksnim bolestima (Borovečki, Fran, MZOS ) ( CroRIS)

Ustanove:
Medicinski fakultet, Zagreb

Profili:

Avatar Url Fran Borovečki (autor)

Avatar Url Dimitri Krainc (autor)

Poveznice na cjeloviti tekst rada:

doi

Citiraj ovu publikaciju:

Cui, Libin; Jeong, Hyun; Borovečki, Fran; Parkhurst, C.N.; Tanese, Naoko; Krainc, Dimitri
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration // Cell, 127 (2006), 1; 59-69 doi:10.1016/j.cell.2006.09.015 (međunarodna recenzija, članak, znanstveni)
Cui, L., Jeong, H., Borovečki, F., Parkhurst, C., Tanese, N. & Krainc, D. (2006) Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell, 127 (1), 59-69 doi:10.1016/j.cell.2006.09.015.
@article{article, author = {Cui, Libin and Jeong, Hyun and Borove\v{c}ki, Fran and Parkhurst, C.N. and Tanese, Naoko and Krainc, Dimitri}, year = {2006}, pages = {59-69}, DOI = {10.1016/j.cell.2006.09.015}, keywords = {energy metabolism, polyglutamine expansion, neurodegeneration}, journal = {Cell}, doi = {10.1016/j.cell.2006.09.015}, volume = {127}, number = {1}, issn = {0092-8674}, title = {Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration}, keyword = {energy metabolism, polyglutamine expansion, neurodegeneration} }
@article{article, author = {Cui, Libin and Jeong, Hyun and Borove\v{c}ki, Fran and Parkhurst, C.N. and Tanese, Naoko and Krainc, Dimitri}, year = {2006}, pages = {59-69}, DOI = {10.1016/j.cell.2006.09.015}, keywords = {energy metabolism, polyglutamine expansion, neurodegeneration}, journal = {Cell}, doi = {10.1016/j.cell.2006.09.015}, volume = {127}, number = {1}, issn = {0092-8674}, title = {Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration}, keyword = {energy metabolism, polyglutamine expansion, neurodegeneration} }

Časopis indeksira:


  • Current Contents Connect (CCC)
  • Web of Science Core Collection (WoSCC)
    • Science Citation Index Expanded (SCI-EXP)
    • SCI-EXP, SSCI i/ili A&HCI
  • Scopus
  • MEDLINE

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