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Mouse Anti-MCCC2 (AA 456-564) Recombinant Antibody (CBFYM-1865) (CBMAB-M2036-FY)

This product is mouse antibody that recognizes MCCC2. The antibody CBFYM-1865 can be used for immunoassay techniques such as: ELISA, IHC-P, WB.
See all MCCC2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-1865
Antibody Isotype
IgG1, k
Application
ELISA, IHC-P, WB

Basic Information

Immunogen
Recombinant protein with GST tag
Specificity
Human
Antibody Isotype
IgG1, k
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
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 456-564

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.
Entrez Gene ID
64087
UniProt ID
Q9HCC0
Alternative Names
Methylcrotonoyl-CoA Carboxylase 2; 3-Methylcrotonyl-CoA Carboxylase Non-Biotin-Containing Subunit; 3-Methylcrotonyl-CoA:Carbon Dioxide Ligase Subunit Beta; Methylcrotonoyl-Coenzyme A Carboxylase 2 (Beta); 3-Methylcrotonyl-CoA Carboxylase 2; MCCase Subunit Beta; EC 6.4.1.4; 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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