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

Name: Mouse Anti-MCEE Recombinant Antibody (6A1)
SKU: CBMAB-G6373-LY
Rating: 5 (1 reviews)

See all MCEE antibodies

Compare Online Inquiry

Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

6A1

Application

IHC-P, WB

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.

More Infomation

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.

Andréasson, M., Zetterström, R. H., von Döbeln, U., Wedell, A., & Svenningsson, P. (2019). MCEE mutations in an adult patient with Parkinson’s disease, dementia, stroke and elevated levels of methylmalonic acid. International journal of molecular sciences, 20(11), 2631.

Heuberger, K., Bailey, H. J., Burda, P., Chaikuad, A., Krysztofinska, E., Suormala, T., ... & Baumgartner, M. R. (2019). Genetic, structural, and functional analysis of pathogenic variations causing methylmalonyl-CoA epimerase deficiency. Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1865(6), 1265-1272.

Ask a question We look forward to hearing from you.

0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Have you used Mouse Anti-MCEE Recombinant Antibody (6A1)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon

$30 eGift Card
Submit a review

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Related Products

Mouse Anti-MCEE Recombinant Antibody (1B5) (CAT#: CBMAB-G6372-LY)

Mouse Anti-MCEE Recombinant Antibody (CBFYM-1866) (CAT#: CBMAB-M2037-FY)

Isotype control

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.).

Online Inquiry

Request bulk or custom quote Second antibody and isotype control

Contact us

Tel: (USA)
(UK)
Fax:

Global Locations

Email:

Online Inquiry