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

Provided herein is a Mouse monoclonal antibody against Human Propionyl-CoA Carboxylase Beta Subunit. The antibody can be used for immunoassay techniques, such as WB, IP, IF, ELISA.
See all PCCB antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
E-7
Antibody Isotype
IgG
Application
WB, IP, IF, ELISA

Basic Information

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.

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
Human5096
Mouse66904
Rat24624
UniProt ID
HumanP05166
MouseQ99MN9
RatP07633
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.
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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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