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Mouse Anti-MCCC1 Recombinant Antibody (CBFYM-0602) (CBMAB-M0735-FY)

This product is mouse antibody that recognizes MCCC1. The antibody CBFYM-0602 can be used for immunoassay techniques such as: ELISA, WB.
See all MCCC1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-0602
Antibody Isotype
IgG2a, k
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a, 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
Buffer
PBS, pH 7.2
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
METHYLCROTONOYL-COA CARBOXYLASE 1
Introduction
This gene encodes the large 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
56922
UniProt ID
Q96RQ3
Alternative Names
Methylcrotonoyl-CoA Carboxylase 1; 3-Methylcrotonyl-CoA Carboxylase Biotin-Containing Subunit; 3-Methylcrotonyl-CoA:Carbon Dioxide Ligase Subunit Alpha; Methylcrotonoyl-Coenzyme A Carboxylase 1 (Alpha); Methylcrotonoyl-CoA Carboxylase Alpha; 3-Methylcrotonyl-CoA Carboxylase 1; MCCase Subunit Alpha
Function
Biotin-attachment 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
Biotin metabolic process Source: UniProtKB
Branched-chain amino acid catabolic process Source: ComplexPortal
Leucine catabolic process Source: ParkinsonsUK-UCL
Cellular Location
Mitochondrion matrix
Involvement in disease
3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D):
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.
PTM
Acetylated.
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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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