Literature summary for 5.4.99.2 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 .

Search for more literature for 5.4.99.2

Application

Application	Comment	Organism
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

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

Localization

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P22033	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
lymphocyte	peripheral lymphocytes	Homo sapiens	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
(R)-methylmalonyl-CoA	-	Homo sapiens	succinyl-CoA	-	r
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

Synonyms	Comment	Organism
MCM	-	Homo sapiens
Methylmalonyl coenzyme A mutase	-	Homo sapiens

Temperature Optimum [°C]

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

pH Optimum

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

Cofactor

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

General Information

General Information	Comment	Organism
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
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

Use of this online version of BRENDA is free under the CC BY 4.0 license. See terms of use for full details.

Release 2023.1 (January 2023)