Protein: ATP-dependent clpX-like chaperone, mitochondrial (EC 3.6.4.10) (ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial) (Caseinolytic mitochondrial matrix peptidase chaperone subunit X) (O76031)
Organism: Homo sapiens (Human)
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.
Subcellular location: Mitochondrion {ECO:0000269|PubMed:11003706}. Mitochondrion matrix, mitochondrion nucleoid {ECO:0000269|PubMed:22841477}.
Sequence: MPSCGACTCGAAAVRLITSSLASAQRGISGGRIHMSVLGRLGTFETQILQRAPLRSFTETPAYFASKDGISKDGSGDGNKKSASEGSSKKSGSGNSGKGGNQLRCPKCGDLCTHVETFVSSTRFVKCEKCHHFFVVLSEADSKKSIIKEPESAAEAVKLAFQQKPPPPPKKIYNYLDKYVVGQSFAKKVLSVAVYNHYKRIYNNIPANLRQQAEVEKQTSLTPRELEIRRREDEYRFTKLLQIAGISPHGNALGASMQQQVNQQIPQEKRGGEVLDSSHDDIKLEKSNILLLGPTGSGKTLLAQTLAKCLDVPFAICDCTTLTQAGYVGEDIESVIAKLLQDANYNVEKAQQGIVFLDEVDKIGSVPGIHQLRDVGGEGVQQGLLKLLEGTIVNVPEKNSRKLRGETVQVDTTNILFVASGAFNGLDRIISRRKNEKYLGFGTPSNLGKGRRAAAAADLANRSGESNTHQDIEEKDRLLRHVEARDLIEFGMIPEFVGRLPVVVPLHSLDEKTLVQILTEPRNAVIPQYQALFSMDKCELNVTEDALKAIARLALERKTGARGLRSIMEKLLLEPMFEVPNSDIVCVEVDKEVVEGKKEPGYIRAPTKESSEEEYDSGVEEEGWPRQADAANS
InterPro domains: - IPR003593: AAA+ ATPase domain (domain) [285-436]
- IPR010603: Zinc finger, ClpX C4-type (domain) [93-146]
- IPR019489: Clp ATPase, C-terminal (domain) [509-603]
- IPR003959: ATPase, AAA-type, core (domain) [285-502]
- IPR027417: P-loop containing nucleoside triphosphate hydrolase (homologous_superfamily) [159-592]
- IPR050052: ATP-dependent Clp protease ATP-binding subunit ClpX (family) [119-603]
- IPR004487: Clp protease, ATP-binding subunit ClpX (family) [276-600]
Interaction partners: - ATP-dependent Clp protease proteolytic subunit, mitochondrial
- Mitochondrial disaggregase
- ATP-dependent zinc metalloprotease YME1L1
- T-box transcription factor TBX22
- Lon protease homolog, mitochondrial
- Mitochondrial inner membrane m-AAA protease component AFG3L2
- GrpE protein homolog 1, mitochondrial
- 60 kDa heat shock protein, mitochondrial
- Mitochondrial inner membrane m-AAA protease component paraplegin
- Lon protease homolog 2, peroxisomal
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0005215 (transporter activity), GO:0140657 (ATP-dependent activity), GO:0016887 (ATP hydrolysis activity), GO:0036094 (small molecule binding), GO:1901363 (heterocyclic compound binding), GO:0016787 (hydrolase activity), GO:0022857 (transmembrane transporter activity), GO:0043167 (ion binding), GO:0097159 (organic cyclic compound binding), GO:0097367 (carbohydrate derivative binding), GO:0005515 (protein binding), GO:0140318 (protein transporter activity), GO:0016817 (hydrolase activity, acting on acid anhydrides), GO:1901265 (nucleoside phosphate binding), GO:0043168 (anion binding), GO:0000166 (nucleotide binding), GO:0032553 (ribonucleotide binding), GO:0035639 (purine ribonucleoside triphosphate binding), GO:0032555 (purine ribonucleotide binding), GO:0016818 (hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides), GO:0017076 (purine nucleotide binding), GO:0016462 (pyrophosphatase activity), GO:0005524 (ATP binding), GO:0032559 (adenyl ribonucleotide binding), GO:0030554 (adenyl nucleotide binding), GO:0017111 (ribonucleoside triphosphate phosphatase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0065007 (biological regulation), GO:0050789 (regulation of biological process), GO:0009987 (cellular process), GO:0048519 (negative regulation of biological process), GO:0019222 (regulation of metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0050794 (regulation of cellular process), GO:0071840 (cellular component organization or biogenesis), GO:0071704 (organic substance metabolic process), GO:0048523 (negative regulation of cellular process), GO:0044238 (primary metabolic process), GO:0009892 (negative regulation of metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0060548 (negative regulation of cell death), GO:0010941 (regulation of cell death), GO:0010605 (negative regulation of macromolecule metabolic process), GO:0019538 (protein metabolic process), GO:0060255 (regulation of macromolecule metabolic process), GO:0016043 (cellular component organization), GO:0043170 (macromolecule metabolic process), GO:0036211 (protein modification process), GO:0043412 (macromolecule modification), GO:0043069 (negative regulation of programmed cell death), GO:0043067 (regulation of programmed cell death), GO:0006996 (organelle organization), GO:0010467 (gene expression), GO:0010629 (negative regulation of gene expression), GO:0010468 (regulation of gene expression), GO:0007005 (mitochondrion organization), GO:0042981 (regulation of apoptotic process), GO:0007006 (mitochondrial membrane organization), GO:0043066 (negative regulation of apoptotic process), GO:0006874 (intracellular calcium ion homeostasis), GO:0018193 (peptidyl-amino acid modification), GO:0007007 (inner mitochondrial membrane organization), GO:0018105 (peptidyl-serine phosphorylation), GO:0018209 (peptidyl-serine modification)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0031975 (envelope), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0031090 (organelle membrane), GO:0031967 (organelle envelope), GO:0005739 (mitochondrion), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0005740 (mitochondrial envelope), GO:0019866 (organelle inner membrane), GO:0043231 (intracellular membrane-bounded organelle), GO:0031966 (mitochondrial membrane), GO:0005743 (mitochondrial inner membrane)