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Literature summary extracted from

  • Gotoh, K.; Nakajima, Y.; Tajima, G.; Hotta, Y.; Kataoka, T.; Kawade, Y.; Sugiyama, N.; Ito, T.; Kimura, K.; Maeda, Y.
    Assay for methylmalonyl coenzyme A mutase activity based on determination of succinyl coenzyme A by ultrahigh-performance liquid chromatography tandem mass spectrometry (2015), Anal. Bioanal. Chem., 407, 5281-5286 .
    View publication on PubMed

Application

EC Number Application Comment Organism
5.4.99.2 analysis establishment of a method to measure the concentration of succinyl-CoA with UPLC-MS/MS after enzyme reaction using peripheral lymphocytes, and investigation of the MCM enzyme activity of patients with methylmalonic acidemia Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
5.4.99.2 L692P naturally occuring mutation, from patient with methylmalonic acidemia (MMA) Homo sapiens
5.4.99.2 R108H naturally occuring mutation, from patient with methylmalonic acidemia (MMA) Homo sapiens
5.4.99.2 R228Q naturally occuring mutation, from patient with methylmalonic acidemia (MMA) Homo sapiens
5.4.99.2 R369H naturally occuring mutation, from patient with methylmalonic acidemia (MMA) Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
5.4.99.2 mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
5.4.99.2 (R)-methylmalonyl-CoA Homo sapiens
-
 succinyl-CoA
-
 r

Organism

EC Number Organism UniProt Comment Textmining
5.4.99.2 Homo sapiens P22033
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
5.4.99.2 lymphocyte peripheral lymphocytes Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
5.4.99.2 (R)-methylmalonyl-CoA
-
 Homo sapiens succinyl-CoA
-
 r
5.4.99.2 additional information establishment of a method to measure the concentration of succinyl-CoA with UPLC-MS/MS after enzyme reaction using peripheral lymphocytes, and investigation of the MCM activity of patients with methylmalonic acidemia, overview. usage of peripheral lymphocytes and withmethylmalonyl-CoA and adenosylcobalamin for the reaction, and analysis of acyl-CoAs by UPLC-MS/MS, structure and cleavage of succinyl-CoA and methylmalonyl-CoA in collision-induced dissociation after lymphocyte disruption. The cone voltage is 45 V and the collision energy is 30 eV Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
5.4.99.2 MCM
-
 Homo sapiens
5.4.99.2 Methylmalonyl coenzyme A mutase
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
5.4.99.2 37
-
 assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
5.4.99.2 7.5
-
 assay at Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
5.4.99.2 adenosylcobalamin active form of vitamin B12 Homo sapiens

General Information

EC Number General Information Comment Organism
5.4.99.2 malfunction methylmalonic acidemia (MMA) is an inherited metabolic disease that is caused by either low MCM activity or deficiency of adenosylcobalamin owing to altered vitamin B12 metabolism Homo sapiens
5.4.99.2 physiological function methylmalonyl coenzyme A (CoA) is a catabolite of valine, isoleucine, methionine, threonine, odd-chain fatty acids, and cholesterol, and is converted to succinyl-CoA by enzymatic reaction of methylmalonyl-CoA mutase (MCM) with its coenzyme vitamin B12 Homo sapiens