Clpp Gene Summary [Mouse]
Predicted to enable ATPase binding activity; identical protein binding activity; and peptidase activity. Predicted to be involved in membrane protein proteolysis and protein quality control for misfolded or incompletely synthesized proteins. Located in mitochondrion. Is expressed in several structures, including early conceptus; heart; liver; pancreas; and placenta. Used to study Perrault syndrome. Human ortholog(s) of this gene implicated in Perrault syndrome. Orthologous to human CLPP (caseinolytic mitochondrial matrix peptidase proteolytic subunit). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Clpp |
| Official Name | caseinolytic mitochondrial matrix peptidase proteolytic subunit [Source:MGI Symbol;Acc:MGI:1858213] |
| Ensembl ID | ENSMUSG00000002660 |
| Bio databases IDs | |
| Aliases | caseinolytic mitochondrial matrix peptidase proteolytic subunit |
| Synonyms | caseinolytic mitochondrial matrix peptidase proteolytic subunit, D17Wsu160e, DFNB81, PRLTS3 |
| Species | Mouse, Mus musculus |
| 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 mouse Clpp often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- endopeptidase
- Clp protease
- crotonase-like
- serine endopeptidase
- peptidase
- endopeptidase Clp
- handle region
- protein binding
- ATP-dependent peptidase
- identical protein binding
Pathways
Biological processes and signaling networks where the Clpp gene in mouse plays a role, providing insight into its function and relevance in health or disease.
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- Perrault syndrome type 1
- Perrault syndrome type 3
- insulin resistance
- impaired glucose tolerance
- obesity
- hepatic steatosis
role in cell
- expression in
- apoptosis
- proliferation
- transmembrane potential
- formation
- size
- fission
- accumulation in
- generation in
- degradation in
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
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Clpp gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- mitochondrial protein catabolic process
- misfolded or incompletely synthesized protein catabolic process
- membrane protein proteolysis
- proteolysis
- proteolysis involved in cellular protein catabolic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
- endopeptidase Clp complex
Molecular Function
What the gene product does at the molecular level
- identical protein binding
- protein binding
- endopeptidase activity
- serine-type endopeptidase activity
- ATP-dependent peptidase activity
- peptidase activity
- ATPase binding