Recombinant Human ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial (CLPX)

Uniprot Id

O76031

Target Species

Human

Target Name

CLPX

Target Full Name

ATP-dependent clpX-like chaperone, mitochondrial

Target 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 upregulation of heme biosynthesis

Target Subcellular Location

Mitochondrion. Mitochondrion matrix, mitochondrion nucleoid.

Target Protein Families

ClpX chaperone family

Target Tissue Specificity

Higher expression in skeletal muscle and heart and to a lesser extent in liver, brain, placenta, lung, kidney and pancreas.

Target Synonyms

ATP dependent Clp protease ATP binding subunit clpX like; mitochondrial; ATP-dependent Clp protease ATP-binding subunit clpX-like; mitochondrial; AU014732; Caseinolytic mitochondrial matrix peptidase chaperone subunit; Caseinolytic peptidase X (E.coli); Caseinolytic protease X; clpX; ClpX caseinolytic peptidase X homolog; ClpX caseinolytic protease X homolog; CLPX_HUMAN; E330029I21; Energy dependent regulator of proteolysis