MCCC1 antibody (AA 104-715)

Catalog No. ABIN7599657

This anti-MCCC1 antibody is a Rabbit Polyclonal antibody detecting MCCC1 in WB, ELISA, IHC, ICC and IF. Suitable for Human, Mouse and Rat.

Quick Overview for MCCC1 antibody (AA 104-715) (ABIN7599657)

Target: MCCC1 (Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This MCCC1 antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF)

Product Details anti-MCCC1 Antibody

Binding Specificity: AA 104-715

Purpose: Anti-MCCC1 Antibody Picoband®

Cross-Reactivity (Details): No cross-reactivity with other proteins.

Characteristics: Anti-MCCC1 Antibody Picoband® (ABIN7599657). 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.

Purification: Immunogen affinity purified.

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

Isotype: IgG

Application Details

Application Notes: Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
Immunohistochemistry, 2-5 μg/mL, Human
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
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.

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

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

Storage: 4 °C,-20 °C

Storage Comment: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Target Details for MCCC1

Target: MCCC1 (Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1))

Alternative Name: MCCC1

Background:

Synonyms: BAG family molecular chaperone regulator 5,BAG-5,Bcl-2-associated athanogene 5,BAG5,KIAA0873,

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

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.

Molecular Weight: 80 kDa

Gene ID: 56922