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| Cat.No | ACP10686 | Target Name | CLPB |
|---|---|---|---|
| Form | Lyophilized powder | Expression System | Custom Production. Please inquire and provide the desire expression system. |
| Expression Range | 1-707 | Protein Length | Full length protein |
| Purity | >85% (SDS-PAGE) | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | Q9H078 |
|---|
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.
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