Dynamic partnership between TFIIH, PGC-1α and SIRT1 is impaired in trichothiodystrophy.

ABSTRACT: The expression of protein-coding genes requires the selective role of many transcription factors, whose coordinated actions remain poorly understood. To further grasp the molecular mechanisms that govern transcription, we focused our attention on the general transcription factor TFIIH, which gives rise, once mutated, to Trichothiodystrophy (TTD), a rare autosomal premature-ageing disease causing inter alia, metabolic dysfunctions. Since this syndrome could be connected to transcriptional defects, we investigated the ability of a TTD mouse model to cope with food deprivation, knowing that energy homeostasis during fasting involves an accurate regulation of the gluconeogenic genes in the liver. Abnormal amounts of gluconeogenic enzymes were thus observed in TTD hepatic parenchyma, which was

SUBMITTER: Traboulsi H 

PROVIDER: S-EPMC4207666 | biostudies-literature | 2014 Oct

REPOSITORIES: biostudies-literature