MCM; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase, mitochondrial

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

E Coli

> 95%

Freeze-dried powder

Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of MUT recombinant protein 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.

Recombinant Protein

Positive Control, Immunogen, SDS-PAGE, Western Blot (WB)

29.8kDa

methylmalonyl-CoA mutase

methylmalonyl-CoA mutase, mitochondrial

Methylmalonyl-CoA mutase, mitochondrial

MCM??[Similar Products]

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

Chromosomal Location of Human Ortholog: 6p12.3

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: methylmalonyl-CoA mutase activity

Biological Process: cobalamin metabolic process; homocysteine metabolic process; short-chain fatty acid catabolic process

Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency

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