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Mouse anti-Human/Mouse/Rat MMUT Monoclonal Antibody

Mouse anti-Human/Mouse/Rat MMUT Monoclonal Antibody — recombinant protein.

SKU: BCREC-000184MA Category:
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Product Specifications

Uniprot No.P22033
Target NamesMMUT
Species ReactivityMouse
ImmunogenRecombinant Human Methylmalonyl-CoA mutase, mitochondrial protein (31-748AA)
ConjugateNon-conjugated
IsotypeIgG1
ClonalityMonoclonal
ApplicationsELISA,WB
BufferPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
StorageUpon receipt, store at -20°C or -80°C. Avoid repeated freeze.

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 Context

Subcellular Location: Mitochondrion matrix; Mitochondrion; Cytoplasm
Disease Association: Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency (MAMM) : 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. MAMM is unresponsive to vitamin B12 therapy. [The disease is caused by variants affecting the gene represented in this entry]

Product Specifications

Mouse anti-Human/Mouse/Rat MMUT Monoclonal Antibody is a recombinant protein. Suitable for ELISA and Western Blot applications.

Applications: ELISA,WB

Biological Activity: Not implied unless activity data are explicitly listed on this product page, the datasheet, or the CoA.

Storage: Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Shipping & Lead Time: Availability, shipping conditions, and estimated lead time are confirmed in the formal quotation or order confirmation based on the product format, destination, and order requirements.

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Protein Biology

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

Subcellular Location

Mitochondrion matrix; Mitochondrion; Cytoplasm

Disease Association

Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency (MAMM) : 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. MAMM is unresponsive to vitamin B12 therapy. [The disease is caused by variants affecting the gene represented in this entry]

Subunit

Homodimer (PubMed:20876572). Interacts (the apoenzyme form) with MMAA; the interaction is GTP dependent (PubMed:20876572, PubMed:21138732, PubMed:28943303)

Gene: MMUT  |  Organism: Homo sapiens  |  Synonyms: Methylmalonyl-CoA isomerase
Key Publications

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Frequently Asked Questions

How do I order or inquire about this product?

Submit the Online Inquiry form with your required quantity and specifications, or email sales@biocrestsci.com. Available sizes, pricing, stock status, documentation, and estimated lead time are confirmed in the formal quotation.

What is the shipping and delivery time?

Availability, dispatch timing, shipping conditions, and delivery estimates depend on the product format, destination, and order requirements. These details are confirmed in the formal quotation or order confirmation.

How should this product be stored?

Follow the storage and handling instructions in the product-specific datasheet and Certificate of Analysis (CoA). Requirements may vary by product form and formulation; contact BioCrest Sci if product-specific guidance is needed.

What quality controls are performed on your products?

Quality-control data such as identity, purity, endotoxin level, or biological activity are provided only when specified on this product page, the datasheet, or Certificate of Analysis (CoA). Biological activity is not implied unless explicitly stated.

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