Human methylmalonyl Coenzyme A mutase ELISA Kit | MUT elisa kit

Human Methylmalonyl-CoA mutase, mitochondrial, MUT ELISA Kit

Cat. Num. AAA9322435

• Gene Names: MUT; MCM
• Reactivity: Human
• Synonyms: methylmalonyl Coenzyme A mutase; Human Methylmalonyl-CoA mutase; mitochondrial; MUT ELISA Kit; mitochondrial (MUT) ELISA kit; MCM; ; MUT elisa kit

• Reactivity: Human
• Specificity: No significant cross-reactivity or interference between Human MCM and analogues was observed.

• Samples: Serum, Plasma, Tissue Homogenate, Feces and Urine
• Assay Type: Sandwich
• Detection Range: 0.625ng/ml-20ng/ml.
• Sensitivity: 0.1ng/ml.
• Intended Uses: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic applications! It is intended to be determinated MCM concentrations in Human serum, plasma and other body fluids. Using Purified Human MCM antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add MCM and MCM antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of MCM in the samples is then determined by comparing the O.D. of the samples to the standard curve.
• Intra-assay Precision: Intra-assay CV (%) is less than 15%.
• Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100]
• Preparation and Storage: Store all reagents at 2-8 degree C

Related Product Information for MUT elisa kit

Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only! It is intended to be determinated MCM concentrations in Human serum, plasma and other body fluids. Using Purified Human MCM antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add MCM and MCM antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of MCM in the samples is then determined by comparing the O.D. of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 156105689
• NCBI GeneID: 4594
• NCBI Accession #: NP_000246.2
• NCBI GenBank Nucleotide #: NM_000255.3
• UniProt Accession #: P22033; Q5SYZ3; Q96B11; Q9UD64; A8K953
• Molecular Weight: 83,134 Da
• NCBI Official Full Name: methylmalonyl-CoA mutase, mitochondrial
• NCBI Official Synonym Full Names: methylmalonyl CoA mutase
• NCBI Official Symbol: MUT
• NCBI Official Synonym Symbols: MCM
• NCBI Protein Information: methylmalonyl-CoA mutase, mitochondrial; methylmalonyl-CoA isomerase; methylmalonyl Coenzyme A mutase
• UniProt Protein Name: Methylmalonyl-CoA mutase, mitochondrial
• UniProt Gene Name: MUT
• UniProt Synonym Gene Names: MCM
• UniProt Entry Name: MUTA_HUMAN

NCBI Description

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]

Uniprot Description

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

Chromosomal Location of Human Ortholog: 6p12.3

Cellular Component: mitochondrion; mitochondrial matrix

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

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

Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency