Clpb Gene Summary [Mouse]
Predicted to enable ATP hydrolysis activity and ATP-dependent protein disaggregase activity. Predicted to be involved in several processes, including RIG-I signaling pathway; antiviral innate immune response; and cellular response to heat. Located in mitochondrion. Is expressed in several structures, including central nervous system; inner cell mass; limb; paravertebral ganglion; and retina. Human ortholog(s) of this gene implicated in 3-methylglutaconic aciduria type 7a; 3-methylglutaconic aciduria type 7b; and severe congenital neutropenia. Orthologous to human CLPB (ClpB family mitochondrial disaggregase). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Retained Intron |
| Official Symbol | Clpb |
| Official Name | ClpB caseinolytic peptidase B [Source:MGI Symbol;Acc:MGI:1100517] |
| Ensembl ID | ENSMUSG00000001829 |
| Bio databases IDs | |
| Aliases | ClpB caseinolytic peptidase B |
| Synonyms | ANKCLB, ANKCLP, ClpB caseinolytic peptidase B, ClpB family mitochondrial disaggregase, HSP78, MEGCANN, MGCA7, MGCA7A, SCN9, SKD3 |
| 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 Clpb often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- transcription regulator
- Ankyrin repeats (many copies)
- Ankyrin repeats (3 copies)
- protein binding
- AAA domain (Cdc48 subfamily)
- ATPase
- Ankyrin repeat
- C-terminal, D2-small domain, of ClpB protein
- AAA domain
- AAA domain (dynein-related subfamily)
- ATPase family associated with various cellular activities (AAA)
- P-loop containing Nucleoside Triphosphate Hydrolases
- ATPases associated with a variety of cellular activities
- ankyrin repeats
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- hereditary disorder
- 3-methylglutaconic aciduria with cataracts, neurologic involvement, and neutropenia
- autosomal dominant severe congenital neutropenia type 9
- severe congenital neutropenia
- 3-methylglutaconic aciduria type VIIA
- idiopathic scoliosis
- Leigh syndrome
role in cell
- apoptosis
- function
- differentiation
- response by
- function in
- synthesis in
- tubulation
- tubulation in
- fragmentation in
- fragmentation
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Nucleus
- Mitochondria
- mitochondrial intermembrane space
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Clpb gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- cellular response to heat
- RIG-I signaling pathway
- granulocyte differentiation
Cellular Component
Where in the cell the gene product is active
- mitochondrial intermembrane space
- cytoplasm
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- ATPase activity
- ATP binding
- protein binding