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Mouse Anti-MUT Recombinant Antibody (CBFYM-2835) (CBMAB-M3028-FY)

This product is mouse antibody that recognizes MUT. The antibody CBFYM-2835 can be used for immunoassay techniques such as: WB, IF.
See all MUT antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-2835
Antibody Isotype
IgG1
Application
WB, IF

Basic Information

Immunogen
Full length human recombinant protein of human MUT(NP_000246) produced in HEK293T cell
Specificity
Human
Antibody Isotype
IgG1
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 7.3, 1% BSA
Preservative
0.02% Sodium azide
Concentration
1 mg/mL
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
METHYLMALONYL-COA MUTASE
Introduction
This gene encodes the mitochondrial enzyme methylmalonyl Coenzyme A mutase. In humans, the product of this gene is a vitamin B12-dependent enzyme which catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, while in other species this enzyme may have different functions. Mutations in this gene may lead to various types of methylmalonic aciduria.
Entrez Gene ID
4594
UniProt ID
P22033
Alternative Names
Methylmalonyl-CoA Mutase; Methylmalonyl Coenzyme A Mutase; Methylmalonyl-CoA Isomerase; EC 5.4.99.2; MCM; Methylmalonyl-CoA Mutase, Mitochondrial; Truncated Methylmalonyl CoA Mutase; Mutant Methylmalonyl CoA Mutase
Function
Catalyzes the reversible isomerization of methylmalonyl-CoA (MMCoA) (generated from branched-chain amino acid metabolism and degradation of dietary odd chain fatty acids and cholesterol) to succinyl-CoA (3-carboxypropionyl-CoA), a key intermediate of the tricarboxylic acid cycle.
Biological Process
Homocysteine metabolic process Source: UniProtKB
Positive regulation of GTPase activity Source: UniProtKB
Post-embryonic development Source: Ensembl
Cellular Location
Cytoplasm
Mitochondrion
Mitochondrion matrix
Involvement in disease
Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency (MMAM):
An often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy.
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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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