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

Name: Mouse Anti-MUT Recombinant Antibody (CBFYM-2835)
SKU: CBMAB-M3028-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

CBFYM-2835

Application

WB, IF

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.

More Infomation

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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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

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.).

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