Aliases for CLPB Gene
- Caseinolytic Mitochondrial Matrix Peptidase Chaperone Subunit B 2 3 5
- Suppressor Of Potassium Transport Defect 3 2 3 4
- HSP78 2 3 4
- SKD3 2 3 4
- ClpB Homolog, Mitochondrial AAA ATPase Chaperonin 2 3
- Ankyrin-Repeat Containing Bacterial Clp Fusion 2 3
- Caseinolytic Peptidase B Protein Homolog 3 4
- ANKCLB 2 3
External Ids for CLPB Gene
Previous GeneCards Identifiers for CLPB Gene
This gene belongs to the ATP-ases associated with diverse cellular activities (AAA+) superfamily. Members of this superfamily form ring-shaped homo-hexamers and have highly conserved ATPase domains that are involved in various processes including DNA replication, protein degradation and reactivation of misfolded proteins. All members of this family hydrolyze ATP through their AAA+ domains and use the energy generated through ATP hydrolysis to exert mechanical force on their substrates. In addition to an AAA+ domain, the protein encoded by this gene contains a C-terminal D2 domain, which is characteristic of the AAA+ subfamily of Caseinolytic peptidases to which this protein belongs. It cooperates with Hsp70 in the disaggregation of protein aggregates. Allelic variants of this gene are associated with 3-methylglutaconic aciduria, which causes cataracts and neutropenia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2015]
GeneCards Summary for CLPB Gene
CLPB (Caseinolytic Mitochondrial Matrix Peptidase Chaperone Subunit B) is a Protein Coding gene. Diseases associated with CLPB include 3-Methylglutaconic Aciduria, Type Vii and 3-Methylglutaconic Aciduria. Among its related pathways are Longevity regulating pathway. Gene Ontology (GO) annotations related to this gene include transcription factor binding and kinase activity.
UniProtKB/Swiss-Prot Summary for CLPB Gene
May function as a regulatory ATPase and be related to secretion/protein trafficking process. Involved in mitochondrial-mediated antiviral innate immunity, activates RIG-I-mediated signal transduction and production of IFNB1 and proinflammatory cytokine IL6 (PubMed:31522117).