5.4.99.2: methylmalonyl-CoA mutase
This is an abbreviated version!
For detailed information about methylmalonyl-CoA mutase, go to the full flat file.
Word Map on EC 5.4.99.2
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5.4.99.2
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methylmalonic
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acidemia
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acidurias
-
adenosylcobalamin
-
propionate
-
succinyl-coa
-
inborn
-
b12-dependent
-
propionyl-coa
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adocbl
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homocysteine
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methylcobalamin
-
apoenzyme
-
shermanii
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adenosylcobalamin-dependent
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5\'-deoxyadenosylcobalamin
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propionibacterium
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hyperammonemia
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homolysis
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adenosylation
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homocystinuria
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mutases
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propionylcarnitine
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adenosyltransferase
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mmaa
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transcobalamins
-
methylmalonyl-coenzyme
-
propionyl
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adocbl-dependent
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odd-chain
-
megaloblastic
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cobalt-carbon
-
cyanocobalamin
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mmachc
-
cobiialamin
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b12-binding
-
hydroxycobalamin
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medicine
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mecbl
-
cobalamin-binding
-
cinnamonensis
-
5\'-deoxyadenosyl
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isobutyryl-coa
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analysis
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extorquens
- 5.4.99.2
-
methylmalonic
- acidemia
- acidurias
- adenosylcobalamin
- propionate
- succinyl-coa
-
inborn
-
b12-dependent
- propionyl-coa
-
adocbl
- homocysteine
- methylcobalamin
-
apoenzyme
- shermanii
-
adenosylcobalamin-dependent
-
5\'-deoxyadenosylcobalamin
- propionibacterium
-
hyperammonemia
-
homolysis
-
adenosylation
- homocystinuria
- mutases
- propionylcarnitine
-
adenosyltransferase
- mmaa
-
transcobalamins
-
methylmalonyl-coenzyme
-
propionyl
-
adocbl-dependent
-
odd-chain
-
megaloblastic
-
cobalt-carbon
- cyanocobalamin
- mmachc
-
cobiialamin
-
b12-binding
- hydroxycobalamin
- medicine
-
mecbl
-
cobalamin-binding
- cinnamonensis
-
5\'-deoxyadenosyl
- isobutyryl-coa
- analysis
- extorquens
Reaction
Synonyms
(R)-2-methyl-3-oxopropanoyl-CoA CoA-carbonylmutase, (S)-Methylmalonyl-CoA mutase, cobalamin-dependent methylmalonyl-CoA mutase, hMCM, L-methylmalonyl-co-enzyme-A mutase, L-methylmalonyl-CoA mutase, MCB-beta, MCM, MCM-alpha, MCM-beta, mcmB, Methylmalonyl CoA mutase, Methylmalonyl coenzyme A carbonylmutase, Methylmalonyl coenzyme A mutase, methylmalonyl-CoA mutase, Methylmalonyl-CoA-carbonyl mutase, mitochondrial methylmalonyl-CoA mutase, mmcm-1, Msed_0638, Msed_2055, MuT, Mutase, methylmalonyl coenzyme A, Sbm, sleeping beauty mutase
ECTree
5.4.99.2 methylmalonyl-CoA mutaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 5.4.99.2 - methylmalonyl-CoA mutase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
Ethylmalonyl-CoA
(2R)-Methylsuccinyl-CoA
-
reacts about 1000-10000 times moreslowly than the natural substrate
+ a small amount of (2S)-diastereoisomer
?
ethylmalonyl-CoA
methylsuccinyl-CoA
-
reacts about 1000-10000 times moreslowly than the natural substrate
-
?
(R)-2-Methyl-3-oxopropanoyl-CoA
?
-
key position in metabolism of propionic acid
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
Sbm is only known to accept or produce (R)-2-methylmalonyl-CoA
-
-
r
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
MCM forms a complex with MeaB
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
MCM forms a complex with MeaB
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
-
?
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
r
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
-
r
(R)-2-methylmalonyl-CoA
succinyl-CoA
-
-
?
(R)-methylmalonyl-CoA
succinyl-CoA
binding of substrate to the enzyme leads to the formation of an electrostatic interaction between a conserved glutamate side chain and the adenosyl ribose of the adenosylcobalamin cofactor. Residue Glu392 is involved in adenosylcobalamin Co-C bond labilization and catalysis
-
-
?
(R)-methylmalonyl-CoA
succinyl-CoA
A4YEG1; A4YIE3
the enzyme is involved in the 3-hydroxypropionate/4-hydroxybutyrate carbon fixation pathway
-
-
r
(R)-methylmalonyl-CoA
succinyl-CoA
A4YEG1; A4YIE3
the enzyme is involved in the 3-hydroxypropionate/4-hydroxybutyrate carbon fixation pathway
-
-
r
(R)-methylmalonyl-CoA
succinyl-CoA
Pyrococcus horikoshii ATCC 700860 / DSM 12428 / JCM 9974 / NBRC 100139 / OT-3
O74009; O58013
-
-
-
r
methylmalonyl-carba(dethia)-coenzyme A
succinyl-carby(dethia)-CoA
-
-
-
-
?
Succinyl-CoA
?
-
production of propionate from succinate
-
-
?
Succinyl-CoA
?
-
succinyl-CoA is isomerized to methylmalonyl-CoA before incorporation into the macrocyclic lactone of erythromycin
-
-
?
additional information
?
-
-
this enzyme is deficient in methylmalonic acidemia
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-
?
additional information
?
-
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this enzyme is deficient in methylmalonic acidemia
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-
?
additional information
?
-
human MMAA and MUT interact in vitro and in vivo. This interaction is nucleotide-selective for MMAA and apoenzyme-dependent for MUT and is associated with stimulation of MMAA GTPase activity by MUT
-
-
?
additional information
?
-
-
human MMAA and MUT interact in vitro and in vivo. This interaction is nucleotide-selective for MMAA and apoenzyme-dependent for MUT and is associated with stimulation of MMAA GTPase activity by MUT
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-
?
additional information
?
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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
-
-
?
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
-
-
?
additional information
?
-
-
P-loop GTPase MeaB plays an auxiliary role in catalytic reaction. MeaB increases affinity of the mutase for cofactor 5-deoxyadenosylcobalamin twofold
-
-
?
