CLPX Gene Summary [Human]
The protein encoded by this gene is part of a protease found in mitochondria. This protease is ATP-dependent and targets specific proteins for degradation. The protease consists of two heptameric rings of the CLPP catalytic subunit sandwiched between two hexameric rings of the chaperone subunit encoded by this gene. Targeted proteins are unwound by this protein and then passed on to the CLPP subunit for degradation. Two transcript variants, one protein-coding and the other non-protein coding, have been found for this gene. [provided by RefSeq, Nov 2015]
Details
| Type | Protein Coding |
| Official Symbol | CLPX |
| Official Name | caseinolytic mitochondrial matrix peptidase chaperone subunit X [Source:HGNC Symbol;Acc:HGNC:2088] |
| Ensembl ID | ENSG00000166855 |
| Bio databases IDs | |
| Aliases | caseinolytic mitochondrial matrix peptidase chaperone subunit X |
| Synonyms | caseinolytic mitochondrial matrix peptidase chaperone subunit, caseinolytic mitochondrial matrix peptidase chaperone subunit X, E330029I21, EPP2 |
| Species | Human, Homo sapiens |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human CLPX often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- ATP binding
- endopeptidase Clp ATP-binding regulatory subunit (clpX)
- enzyme
- protein binding
- ATP-dependent peptidase
- protease activator
- AAA domain (Cdc48 subfamily)
- ATPase
- C-terminal, D2-small domain, of ClpB protein
- ATP-dependent protease HslVU, ATPase subunit
- AAA domain (dynein-related subfamily)
- Zn-ribbon
- P-loop containing Nucleoside Triphosphate Hydrolases
- ATPases associated with a variety of cellular activities
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- erythropoietic protoporphyria type 2
- bipolar disorder
role in cell
- degradation in
- expression in
- production in
- binding in
- phosphorylation in
- josamycin sensitivity
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Mitochondria
- cytosol
- mitochondrial matrix
- mitochondrial nucleoids
- mitochondrial inner membrane
- nucleoplasm
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the human CLPX gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- proteolysis
- ATP metabolic process
- protein folding
- proteolysis involved in cellular protein catabolic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
- cytosol
- endopeptidase Clp complex
- mitochondrial endopeptidase Clp complex
- mitochondrial inner membrane
- mitochondrial nucleoid
- nucleoplasm
Molecular Function
What the gene product does at the molecular level
- ATPase activity
- ATP binding
- zinc ion binding
- peptidase activator activity
- protein binding
- protein dimerization activity
- unfolded protein binding
- ATP-dependent peptidase activity