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Mouse Anti-MCEE Recombinant Antibody (6A1) (CBMAB-G6373-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
6A1
Antibody Isotype
IgG2a
Application
IHC-P, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
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
50% glycerol, 1% BSA
Preservative
0.02% sodium azide
Concentration
1 mg/mL
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
METHYLMALONYL-COA EPIMERASE
Introduction
The product of this gene catalyzes the interconversion of D- and L-methylmalonyl-CoA during the degradation of branched chain amino acids. odd chain-length fatty acids, and other metabolites. Mutations in this gene result in methylmalonyl-CoA epimerase deficiency, which is presented as mild to moderate methylmalonic aciduria. [provided by RefSeq, Jul 2008]
Entrez Gene ID
84693
UniProt ID
Q96PE7
Alternative Names
Methylmalonyl-CoA Epimerase; Glyoxalase Domain Containing 2; DL-Methylmalonyl-CoA Racemase; Methylmalonyl-CoA Epimerase; Mitochondrial; EC 5.1.99.1; GLOD2;
Function
Methylmalonyl-CoA epimerase involved in propionyl-CoA metabolism.
Biological Process
L-methylmalonyl-CoA metabolic process Source: UniProtKB
Short-chain fatty acid catabolic process Source: Reactome
Cellular Location
Mitochondrion
Involvement in disease
Methylmalonyl-CoA epimerase deficiency (MCEED):
Autosomal recessive inborn error of amino acid metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria may present in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma.
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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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