PCCB Antibody (E-12): sc-393929

PCCB Antibody (E-12) is a mouse monoclonal IgG2a kappa light chain antibody that detects PCCB protein of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). PCCB (E-12) 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. PCCB protein, or Propionyl Coenzyme A carboxylase beta chain, is a crucial component of the propionyl-CoA carboxylase complex, which plays a vital role in the metabolism of certain amino acids and odd-chain fatty acids. Specifically, PCCB catalyzes the biotin-dependent carboxylation of propionyl-CoA to d-methylmalonyl-CoA, a key step in the metabolic pathway that converts propionyl-CoA into succinyl-CoA, which can then enter the citric acid cycle for energy production. Proper functioning of PCCB is essential for maintaining metabolic homeostasis, and inherited mutations in the PCCB gene can lead to propionic acidemia type II, a serious autosomal recessive disorder characterized by a range of symptoms including metabolic crises, developmental delays, and immune deficiencies. Understanding the structure and function of PCCB is critical for developing therapeutic strategies for managing this condition and for advancing research in metabolic disorders.

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

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

PCCB Antibody (E-12) References:

1. Changes in the carboxyl terminus of the beta subunit of human propionyl-CoA carboxylase affect the oligomer assembly and catalysis: expression and characterization of seven patient-derived mutant forms of PCC in Escherichia coli.  |  Chloupková, M., et al. 2000. Mol Genet Metab. 71: 623-32. PMID: 11136555
2. Propionyl-CoA and adenosylcobalamin metabolism in Caenorhabditis elegans: evidence for a role of methylmalonyl-CoA epimerase in intermediary metabolism.  |  Chandler, RJ., et al. 2006. Mol Genet Metab. 89: 64-73. PMID: 16843692
3. New splicing mutations in propionic acidemia.  |  Desviat, LR., et al. 2006. J Hum Genet. 51: 992-997. PMID: 17051315
4. Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes.  |  Lamhonwah, AM., et al. 1986. Proc Natl Acad Sci U S A. 83: 4864-8. PMID: 3460076
5. Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit.  |  Lamhonwah, AM., et al. 1994. Genomics. 19: 500-5. PMID: 8188292
6. The molecular defect in propionic acidemia: exon skipping caused by an 8-bp deletion from an intron in the PCCB allele.  |  Ohura, T., et al. 1993. Hum Genet. 92: 397-402. PMID: 8225321
7. Three independent mutations in the same exon of the PCCB gene: differences between Caucasian and Japanese propionic acidaemia.  |  Tahara, T., et al. 1993. J Inherit Metab Dis. 16: 353-60. PMID: 8411997
8. Human propionyl-CoA carboxylase beta subunit gene: exon-intron definition and mutation spectrum in Spanish and Latin American propionic acidemia patients.  |  Rodríguez-Pombo, P., et al. 1998. Am J Hum Genet. 63: 360-9. PMID: 9683601

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-PCCB Antibody (E-12) has 2 citations in a variety of scientific publications.

Primary antibodies like Anti-PCCB Antibody (E-12) 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.

PCCB Antibody (E-12) Specifications

Alternate Product Name: PCCB

Product Clone Name: E-12

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 240-539

Epitope Target: PCCB

Epitope Species: human

Product Reactivity/Detection: PCCB

Species Reactivity/Detection: 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 PCCB Antibody (E-12): 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