Mouse Anti-PCCB Recombinant Antibody (E-7) (CBMAB-P0954-YC)

Name: Mouse Anti-PCCB Recombinant Antibody (E-7)
SKU: CBMAB-P0954-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

E-7

Application

WB, IP, IF, ELISA

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

Propionyl-CoA Carboxylase Beta Subunit

Introduction

PCCB is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2).

Entrez Gene ID

Human	5096
Mouse	66904
Rat	24624

UniProt ID

Human	P05166
Mouse	Q99MN9
Rat	P07633

Alternative Names

Propionyl-CoA Carboxylase Beta Subunit; EC 6.4.1.3; Propionyl Coenzyme A Carboxylase, Beta Polypeptide; Propanoyl-CoA:Carbon Dioxide Ligase Subunit Beta; PCCase Subunit Beta;

Function

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed:6765947, PubMed:15890657).
Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:6765947, PubMed:15890657).
Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity).
Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate (PubMed:6765947).
Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).

Biological Process

Branched-chain amino acid metabolic process1 PublicationIC:ComplexPortal
Fatty acid metabolic process1 PublicationIC:ComplexPortal
Short-chain fatty acid catabolic process1 PublicationIC:UniProtKB

Cellular Location

Mitochondrion matrix

Involvement in disease

Propionic acidemia type II (PA-2):
Life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.

Han, H. V., Efem, R., Rosati, B., Lu, K., Maimouni, S., Jiang, Y. P., ... & Lin, R. Z. (2023). Propionyl-CoA carboxylase subunit B regulates anti-tumor T cells in a pancreatic cancer mouse model. bioRxiv, 2023-07.

Komachali, S. R., Siahpoosh, Z., Komachali, S. R., Tamandani, D. M. K., & Salehi, M. (2022). Novel mutations of PCCA and PCCB genes found by whole-exome sequencing related to propionic acidemia patients. European Journal of Biological Research, 12(4), 294-306.

Mamoor, S. (2021). Over-expression of propionyl-CoA carboxylase beta subunit in human endometrial cancer.

Yang, X., Li, D., Tu, C., He, W., Meng, L., Tan, Y. Q., ... & Zhang, Q. (2021). Novel variants of the PCCB gene in Chinese patients with propionic acidemia. Clinica Chimica Acta, 519, 18-25.

Pournasr, B., & Duncan, S. A. (2020). Generation of isogenic Propionyl-CoA carboxylase beta subunit (PCCB) deficient induced pluripotent stem cell lines. Stem cell research, 48, 101953.

Yang, Q., Xu, H., Luo, J., Li, M., Yi, S., Zhang, Q., ... & Fan, X. (2020). three novel variants in PCCA and PCCB genes in Chinese patients with propionic acidemia. BMC Medical Genetics, 21, 1-6.

Wenger, O., Brown, M., Smith, B., Chowdhury, D., Crosby, A. H., Baple, E. L., ... & Strauss, K. A. (2020). Biochemical phenotype and its relationship to treatment in 16 individuals with PCCB c. 1606A> G (p. Asn536Asp) variant propionic acidemia. Molecular Genetics and Metabolism, 131(3), 316-324.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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