The fanconi anemia core complex acts as a transcriptional co-regulator in hairy enhancer of split 1 signaling.

ABSTRACT: Mutations in one of the 13 Fanconi anemia (FA) genes cause a progressive bone marrow failure disorder associated with developmental abnormalities and a predisposition to cancer. Although FA has been defined as a DNA repair disease based on the hypersensitivity of patient cells to DNA cross-linking agents, FA patients develop various developmental defects such as skeletal abnormalities, microphthalmia, and endocrine abnormalities that may be linked to transcriptional defects. Recently, we reported that the FA core complex interacts with the transcriptional repressor Hairy Enhancer of Split 1 (HES1) suggesting that the core complex plays a role in transcription. Here we show that the FA core complex contributes to transcriptional regulation of HES1-responsive genes, including HES1 and the cy

SUBMITTER: Tremblay CS 

PROVIDER: S-EPMC2679438 | biostudies-literature | 2009 May

REPOSITORIES: biostudies-literature