Anti-MCCC1 Antibody Picoband®, Dylight594 Conjugate

Boster Bio Anti-MCCC1 Antibody Picoband® catalog # A07441-1. Tested in ELISA, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.

Synonyms

BAG family molecular chaperone regulator 5;BAG-5;Bcl-2-associated athanogene 5;BAG5;KIAA0873;

Gene Name

BAG family molecular chaperone regulator 5

Gene ID

56922

UniProt

Q96RQ3

Host

Rabbit

Reactivity

Human,Mouse,Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E.coli-derived human MCCC1 recombinant protein (Position: Q104-E715).

Clonality

Polyclonal

Tissue Specificity

Expressed in all tissues examined with lower levels in brain and testis.

Applications

WB,IHC,ICC,IF,ELISA

Purification

Immunogen affinity purified.

Concentration

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

Form

Lyophilized

Reconstitution

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

Function

Inhibits both auto-ubiquitination of PARK2 and ubiquitination of target proteins by PARK2 (By similarity). May function as a nucleotide exchange factor for HSP/HSP70, promoting ADP release, and activating Hsp70-mediated refolding. .

Components

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

References & Citations

1. Baumgartner, M. R., Almashanu, S., Suormala, T., Obie, C., Cole, R. N., Packman, S., Baumgartner, E. R., Valle, D. The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. J. Clin. Invest. 107: 495-504, 2001. 2. Baumgartner, M. R., Dantas, M. F., Suormala, T., Almashanu, S., Giunta, C., Friebel, D., Gebhardt, B., Fowler, B., Hoffmann, G. F., Baumgartner, E. R., Valle, D. Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy. Am. J. Hum. Genet. 75: 790-800, 2004. 3. Gallardo, M. E., Desviat, L. R., Rodriguez, J. M., Esparza-Gordillo, J., Perez-Cerda, C., Perez, B., Rodriguez-Pombo, P., Criado, O., Sanz, R., Morton, D. H., Gibson, K. M., Le, T. P., Ribes, A., Rodriguez de Cordoba, S., Ugarte, M., Penalva, M. A. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am. J. Hum. Genet. 68: 334-346, 2001.

Storage Conditions

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 freezing and thawing.

Calculated Molecular Weight

51200 MW

Observed Molecular Weight

75 kDa

Fragment

Rabbit IgG

Applications Notes

Western blot, 0.25-0.5 μg/ml, Human, Rat<br> Immunohistochemistry, 2-5 μg/ml, Human, Mouse, Rat<br> Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human<br> ELISA, 0.1-0.5 μg/ml, -<br>