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Mouse Anti-MCCC2 Recombinant Antibody (2B3) (CBMAB-A5339-LY)

The product is antibody recognizes MCCC2. The antibody 2B3 immunoassay techniques such as: WB, ELISA.
See all MCCC2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2B3
Antibody Isotype
IgG1, κ
Application
WB, ELISA

Basic Information

Immunogen
MCCC2 (NP_071415, 456 a.a. ~ 563 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG1, κ
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!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
Introduction
This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq]
Entrez Gene ID
64087
UniProt ID
Q9HCC0
Alternative Names
MCCB
Function
Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Biological Process
Branched-chain amino acid catabolic process Source: ComplexPortal
Coenzyme A metabolic process Source: Ensembl
Leucine catabolic process Source: GO_Central
Cellular Location
Mitochondrion matrix
Involvement in disease
3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D):
An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.
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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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