Anti-CLPB Antibody Picoband®

SKU: A02912-2

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Monkey,Mouse,Rat

Applications: WB,IHC,ICC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5μg/ml, Human, Mouse, Rat, Monkey
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human
Immunocytochemistry/Immunofluorescence, 4μg/ml, Human
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human
ELISA, 0.1-0.5μg/ml, -

Gene Name: CLPB

Specificity:

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.

Immunogen: E.coli-derived human CLPB recombinant protein (Position: Q438-I707).

Clonality: Polyclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Capo-Chichi, J.-M., Boissel, S., Brustein, E., Pickles, S., Fallet-Bianco, C., Nassif, C., Patry, L., Dobrzeniecka, S., Liao, M., Labuda, D., Samuels, M. E., Hamdan, F. F., Vande Velde, C., Rouleau, G. A., Drapeau, P., Michaud, J. L. Disruption of CLPB is associated with congenital microcephaly, severe encephalopathy and 3-methylglutaconic aciduria. J. Med. Genet. 52: 303-311, 2015. 2. Haslberger, T., Zdanowicz, A., Brand, I., Kirstein, J., Turgay, K., Mogk, A., Bukau, B. Protein disaggregation by the AAA+ chaperone ClpB involves partial threading of looped polypeptide segments. Nature Struct. Molec. Biol. 15: 641-650, 2008. 3. Saunders, C., Smith, L., Wibrand, F., Ravn, K., Bross, P., Thiffault, I., Christensen, M., Atherton, A., Farrow, E., Miller, N., Kingsmore, S. F., Ostergaard, E. CLPB variants associated with autosomal-recessive mitochondrial disorder with cataract, neutropenia, epilepsy, and methylglutaconic aciduria. Am. J. Hum. Genet. 96: 258-265, 2015.

Uniprot ID: Q9H078

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 70 kDa

Calculated Molecular Weight: 136311 MW

Gene ID: 81570

Protein Name: Mitochondrial disaggregase

Gene Full Name: ClpB family mitochondrial disaggregase

Synonyms: CLPB; HSP78; SKD3