Rabbit anti-Human CLPB Polyclonal Antibody

Uniprot Id

Q9H078

Target Species

Human

Target Name

CLPB

Target Full Name

Mitochondrial disaggregase

Target Function

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.

Target Involvement

3-methylglutaconic aciduria with cataracts, neurologic involvement and neutropenia (MEGCANN)

Target Subcellular Location

Mitochondrion intermembrane space.

Target Protein Families

ClpA/ClpB family

Target Tissue Specificity

Widely expressed (at protein level). Expressed in fetal, as well as in adult tissues, with highest levels in adult brain, including thalamus, hippocampus, occipital cortex and parietal cortex. Low expression in granulocytes.

Target Synonyms

Caseinolytic peptidase B; Caseinolytic peptidase B protein homolog; clpB; ClpB caseinolytic peptidase B homolog (E. coli); ClpB caseinolytic peptidase B homolog; CLPB_HUMAN; FLJ13152; HSP78; SKD3; Suppressor of potassium transport defect 3

Target Background

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.