Carbohydrate-responsive element-binding protein

Carbohydrate-responsive element-binding protein (ChREBP) also known as MLX-interacting protein-like (MLXIPL) is a is_associated_with::protein that in humans is encoded by the MLXIPL is_associated_with::gene. The protein name derives from the protein's interaction with carbohydrate response element sequences of DNA.

Function
This gene encodes a is_associated_with::basic helix-loop-helix leucine zipper is_associated_with::transcription factor of the is_associated_with::Myc / Max / Mad superfamily. This protein forms a heterodimeric complex and binds and activates, in a glucose-dependent manner, carbohydrate response element (ChoRE) motifs in the promoters of is_associated_with::triglyceride synthesis genes.

Clinical significance
This gene is deleted in Williams-Beuren syndrome, a multisystem developmental disorder caused by the deletion of contiguous genes at chromosome 7q11.23.

Interactions
MLXIPL has been shown to interact with MLX.

Role in glycolysis
ChREBP is translocated to the nucleus and binds to DNA after dephosphorylation of a p-Ser and a p-Thr residue by is_associated_with::PP2A, which itself is activated by is_associated_with::Xylulose-5-phosphate. Xu5p is produced in the is_associated_with::pentose phosphate pathway when levels of is_associated_with::Glucose-6-phosphate are high (the cell has ample glucose). In the liver, ChREBP mediates activation of several regulatory enzymes of glycolysis and lipogenesis including L-type pyruvate kinase (L-PK), acetyl CoA carboxylase, and fatty acid synthase.