CLPX
Description
The CLPX (caseinolytic mitochondrial matrix peptidase chaperone subunit X) is a protein-coding gene located on chromosome 15.
CLPX is an ATP-dependent specificity component of the Clp protease complex. It hydrolyzes ATP and targets specific substrates for degradation by the Clp complex. CLPX can also act as a chaperone in the absence of CLPP, enhances the DNA-binding activity of TFAM, and is essential for maintaining a normal mitochondrial nucleoid structure. It functions as an ATP-dependent unfoldase, stimulating the incorporation of the pyridoxal phosphate cofactor into 5-aminolevulinate synthase, which activates 5-aminolevulinate (ALA) synthesis, the first step in heme biosynthesis. CLPX plays a vital role in efficient erythropoiesis by up-regulating heme biosynthesis.
CLPX is also known as EPP2.
