MCCB Antibody (B-1): sc-390836

MCCB Antibody (B-1) is a mouse monoclonal IgG2b kappa light chain antibody that detects MCCB protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). MCCB (B-1) 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. Methylcrotonoyl-CoA carboxylase beta chain, or MCCB, is a crucial component of the MCC enzyme complex, which is essential for the catabolism of the branched-chain amino acid leucine. MCCB consists of 563 amino acids and features an N-terminal mitochondrial targeting sequence, indicating localization within the mitochondria, where MCCB plays a vital role in energy metabolism. MCCB forms part of a putative dodecamer structure composed of six biotin-containing alpha subunits and six beta subunits, which is important for enzymatic function. Deficiencies in the MCCC2 gene, which encodes MCCB, can lead to 3-methylcrotonylglycinuria type II (MCGII), a genetic disorder characterized by muscular hypotonia and atrophy, highlighting MCCB′s significance in maintaining metabolic health.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

MCCB Antibody (B-1) 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. Isolated biotin-resistant deficiency of 3-methylcrotonyl-CoA carboxylase presenting as a clinically severe form in a newborn with fatal outcome.  |  Bannwart, C., et al. 1992. J Inherit Metab Dis. 15: 863-8. PMID: 1293382
5. Functional analysis of MCCA and MCCB mutations causing methylcrotonylglycinuria.  |  Desviat, LR., et al. 2003. Mol Genet Metab. 80: 315-20. PMID: 14680978
6. 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
7. 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
8. Molecular mechanism of dominant expression in 3-methylcrotonyl-CoA carboxylase deficiency.  |  Baumgartner, MR. 2005. J Inherit Metab Dis. 28: 301-9. PMID: 15868465
9. Bovine kidney 3-methylcrotonyl-CoA and propionyl-CoA carboxylases: each enzyme contains nonidentical subunits.  |  Lau, EP., et al. 1979. Proc Natl Acad Sci U S A. 76: 214-8. PMID: 16170895
10. Newborn screening for 3-methylcrotonyl-CoA carboxylase deficiency: population heterogeneity of MCCA and MCCB mutations and impact on risk assessment.  |  Stadler, SC., et al. 2006. Hum Mutat. 27: 748-59. PMID: 16835865

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-MCCB Antibody (B-1) has 1 citations in a variety of scientific publications.

Primary antibodies like Anti-MCCB Antibody (B-1) 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.

MCCB Antibody (B-1) Specifications

Alternate Product Name: MCCB

Product Clone Name: B-1

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2b kappa light chain

Amino Acids: 241-540

Epitope Target: MCCB

Epitope Species: human

Product Reactivity/Detection: MCCB

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, IHC(P), ELISA

Vialing Information: Each vial contains 200 µg IgG_2b kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 1 citations in various scientific publications

Available Conjugates/Forms for MCCB Antibody (B-1): 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