| Tissue Specificity | Higher expression in skeletal muscle and heart and to a lesser extent in liver, brain, placenta, lung, kidney and pancreas. |
| Function | 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. |
| Protein Name | Atp-Dependent Clp Protease Atp-Binding Subunit Clpx-Like - Mitochondrial |
| Database Links | Reactome: R-HSA-9837999 |
| Cellular Localisation | MitochondrionMitochondrion MatrixMitochondrion Nucleoid |
| Alternative Antibody Names | Anti-Atp-Dependent Clp Protease Atp-Binding Subunit Clpx-Like - Mitochondrial antibodyAnti-CLPX antibody |
Information sourced from Uniprot.org
