MCCA Antibody (B-7): sc-365754

MCCA Antibody (B-7) is a mouse monoclonal IgG2a kappa light chain antibody that detects MCCA protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). MCCA (B-7) antibody is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. The 3-methylcrotonyl-CoA carboxylase α chain (MCCA) plays a vital role in the metabolism of branched-chain amino acids, particularly leucine, by catalyzing the carboxylation of 3-methylcrotonyl-CoA to produce 3-methylglutaconyl-CoA, a critical step in the energy production pathway. Located in the mitochondrial matrix, MCCA functions as a heterodimer and requires a biotin cofactor for enzymatic activity. The gene encoding the 725 amino acid MCCA protein is situated on chromosome 3q26-q28 and consists of 19 exons. Mutations in this gene can lead to 3-methylcrotonylglycinuria (MCGI), an autosomal recessive disorder that manifests with symptoms such as muscular hypotonia and atrophy, while human MCC deficiency can result in the accumulation of 3-methylcrotonyl-CoA, leading to a spectrum of clinical presentations ranging from asymptomatic cases to severe metabolic acidosis and potential fatality in infancy.

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MCCA Antibody (B-7) References:

1. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism.  |  Gallardo, ME., et al. 2001. Am J Hum Genet. 68: 334-46. PMID: 11170888
2. The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.  |  Baumgartner, MR., et al. 2001. J Clin Invest. 107: 495-504. PMID: 11181649
3. Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency.  |  Holzinger, A., et al. 2001. Hum Mol Genet. 10: 1299-306. PMID: 11406611
4. Fungal metabolic model for 3-methylcrotonyl-CoA carboxylase deficiency.  |  Rodríguez, JM., et al. 2004. J Biol Chem. 279: 4578-87. PMID: 14612443
5. Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy.  |  Baumgartner, MR., et al. 2004. Am J Hum Genet. 75: 790-800. PMID: 15359379
6. Biotin deficiency reduces expression of SLC19A3, a potential biotin transporter, in leukocytes from human blood.  |  Vlasova, TI., et al. 2005. J Nutr. 135: 42-7. PMID: 15623830
7. In vivo protein biotinylation for identification of organ-specific antigens accessible from the vasculature.  |  Rybak, JN., et al. 2005. Nat Methods. 2: 291-8. PMID: 15782212
8. Multiple biotin-containing proteins in 3T3-L1 cells.  |  Chandler, CS. and Ballard, FJ. 1986. Biochem J. 237: 123-30. PMID: 3800873
9. Isolated biotin-resistant 3-methylcrotonyl CoA carboxylase deficiency presenting with life-threatening hypoglycaemia.  |  Bartlett, K., et al. 1984. J Inherit Metab Dis. 7: 182. PMID: 6441868

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-MCCA Antibody (B-7) has 2 citations in a variety of scientific publications.

Primary antibodies like Anti-MCCA Antibody (B-7) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

MCCA Antibody (B-7) Specifications

Alternate Product Name: MCCA

Product Clone Name: B-7

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 436-725

Epitope Target: MCCA

Epitope Species: human

Product Reactivity/Detection: MCCA

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG_2a kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 2 citations in various scientific publications

Available Conjugates/Forms for MCCA Antibody (B-7): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, PE: Phycoerythrin