MLXIPL
Description
The MLXIPL (MLX interacting protein like) is a protein-coding gene located on chromosome 7.
MLXIPL, also known as ChREBP (carbohydrate-responsive element-binding protein), is a protein encoded by the MLXIPL gene in humans. It belongs to the Myc/Max/Mad superfamily of transcription factors and forms a heterodimer complex that binds to carbohydrate response elements (ChoRE) in the promoters of triglyceride synthesis genes. MLXIPL is activated by glucose, independent of insulin, and promotes de novo lipogenesis in adipose tissue and glycolysis and lipogenesis in the liver. Deletions of the MLXIPL gene are associated with Williams-Beuren syndrome, a multisystem developmental disorder. Excess expression of MLXIPL in the liver can contribute to steatosis (fatty liver) in metabolic syndrome and type 2 diabetes.
MLXIPL, also known as ChREBP, forms a heterodimer with MLX/TCFL4 to bind DNA and activate transcription. It binds to the canonical E box sequence 5'-CACGTG-3' and plays a role in activating glycolytic genes. MLXIPL is involved in glucose-responsive gene regulation, adjusting transcription based on cellular carbohydrate levels. During transitions from fasting to feeding, refeeding stimulates MLXIPL/ChREBP, leading to increased expression of BCKDK relative to PPM1K. This shift promotes phosphorylation and activation of ACLY, ultimately producing malonyl-CoA and oxaloacetate, essential substrates for de novo lipogenesis and gluconeogenesis, respectively.
MLXIPL is also known as CHREBP, MIO, MLX, MONDOB, WBSCR14, WS-bHLH, bHLHd14.
