Methylmalonyl-CoA Mutase, Mitochondrial (MCM), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of MCM elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9352552 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Methylmalonyl-CoA Mutase, Mitochondrial (MCM) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MCM. The ELISA analytical biochemical technique of the MBS9352552 kit is based on MCM antibody-MCM antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MCM antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MCM. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

83,134 Da

methylmalonyl-CoA mutase

methylmalonyl-CoA mutase, mitochondrial

Methylmalonyl-CoA mutase, mitochondrial

MCM  [Similar Products]

MUTA_HUMAN

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. [provided by RefSeq, Jul 2008]

MUT: Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM). MMAM is 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. Belongs to the methylmalonyl-CoA mutase family.

Protein type: Carbohydrate Metabolism - propanoate; Isomerase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; EC 5.4.99.2

Chromosomal Location of Human Ortholog: 6p12.3

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: cobalamin binding; metal ion binding; methylmalonyl-CoA mutase activity

Biological Process: cellular lipid metabolic process; cobalamin metabolic process; fatty acid beta-oxidation; homocysteine metabolic process; post-embryonic development; short-chain fatty acid catabolic process; vitamin metabolic process; water-soluble vitamin metabolic process

Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency

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