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mRNA expression levels of PGC-1α in a transgenic and a toxin model of Huntington's disease.

Abstract
Peroxisome proliferator-activated receptor-gamma (PPARγ) coactivator-1 alpha (PGC-1α) is involved in the regulation of mitochondrial biogenesis, respiration, and adaptive thermogenesis. The full-length PGC-1α (FL-PGC-1α) comprises multiple functional domains interacting with several transcriptional regulatory factors such as nuclear respiratory factors, estrogen-related receptors, and PPARs; however, a number of PGC-1α splice variants have also been reported recently. In this study, we examined the expression levels of FL-PGC-1α and N-truncated PGC-1α (NT-PGC-1α), a shorter but functionally active splice variant of PGC-1α protein, in N171-82Q transgenic and 3-nitropropionic acid-induced murine model of Huntington's disease (HD). The expression levels were determined by RT-PCR in three brain areas (striatum, cortex, and cerebellum) in three age groups (8, 12, and 16 weeks). Besides recapitulating prior findings that NT-PGC-1α is preferentially increased in 16 weeks of age in transgenic HD animals, we detected age-dependent alterations in both models, including a cerebellum-predominant upregulation of both PGC-1α variants in transgenic mice, and a striatum-predominant upregulation of both PGC-1α variants after acute 3-nitropropionic acid intoxication. The possible relevance of this expression pattern is discussed. Based on our results, we assume that increased expression of PGC-1α may serve as a compensatory mechanism in response to mitochondrial damage in transgenic and toxin models of HD, which may be of therapeutic relevance.
AuthorsRita Török, Júlia Anna Kónya, Dénes Zádori, Gábor Veres, Levente Szalárdy, László Vécsei, Péter Klivényi
JournalCellular and molecular neurobiology (Cell Mol Neurobiol) Vol. 35 Issue 2 Pg. 293-301 (Mar 2015) ISSN: 1573-6830 [Electronic] United States
PMID25319408 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Nitro Compounds
  • Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha
  • Ppargc1a protein, mouse
  • Propionates
  • RNA, Messenger
  • Transcription Factors
  • 3-nitropropionic acid
Topics
  • Animals
  • Disease Models, Animal
  • Female
  • Gene Expression Regulation
  • Huntington Disease (genetics, pathology)
  • Male
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Nitro Compounds
  • Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha
  • Propionates
  • RNA, Messenger (genetics, metabolism)
  • Transcription Factors (genetics, metabolism)

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