ChREBP Background Information ChREBP (for carbohydrate responsive binding protein, also designated Mlx interactor, WBSCR14 and MondoB) is a transcription factor that binds to the carbohydrate-responsive element of the L-type pyruvate kinase gene (L-PK). ChREBP is expressed specifically in liver and is activated by high glucose and inhibited by cAMP or a high fat diet. ChREBP is likely critical for the optimal long-term storage of excess carbohydrates as fats, and may contribute to the imbalance between nutrient utilization and storage, which is characteristic of obesity. ChREBP represses E-box-dependent transcription forms and forms heterodimers with Mlx to bind the DNA sequence CACGTG. ChREBP is encoded by the WBSCR14 gene, which is located within the Williams-Beuren syndrome (WBS) deletion at chromosome 7q11.23. WBS is a neuro-developmental disorder affecting several systems. Loss of the encoded transcription factor may contribute to the developmental symptoms found in WBS.
