ATP-dependent specificity component of the Clp protease complex. Hydrolyzes ATP . Targets specific substrates for degradation by the Clp complex (, ). Can perform chaperone functions in the absence of CLPP. Enhances the DNA-binding activity of TFAM and is required for maintaining a normal mitochondrial nucleoid structure . ATP-dependent unfoldase that stimulates the incorporation of the pyridoxal phosphate cofactor into 5-aminolevulinate synthase, thereby activating 5-aminolevulinate (ALA) synthesis, the first step in heme biosynthesis . Important for efficient erythropoiesis through up-regulation of heme biosynthesis (, ).
Subcellular locations: Mitochondrion, Mitochondrion matrix, Mitochondrion nucleoid
Higher expression in skeletal muscle and heart and to a lesser extent in liver, brain, placenta, lung, kidney and pancreas.