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Crystal structure of human methylmalonyl-CoA mutase in complex with adenosylcobalamin

Source organism: Homo sapiens
Biochemical function: modified amino acid binding
Biological process: succinyl-CoA biosynthetic process
Cellular component: mitochondrial matrix

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EC 5.4.99.2: Methylmalonyl-CoA mutase

Reaction catalysed:
(R)-methylmalonyl-CoA = succinyl-CoA
Systematic name:
(R)-methylmalonyl-CoA CoA-carbonylmutase
Alternative Name(s):
  • (R)-2-methyl-3-oxopropanoyl-CoA CoA-carbonylmutase
  • (S)-methylmalonyl-CoA mutase
  • Methylmalonyl coenzyme A carbonylmutase
  • Methylmalonyl coenzyme A mutase
  • Methylmalonyl-CoA CoA-carbonyl mutase

GO terms

Sequence families

Pfam Protein families (Pfam)
PF02310
Domain description: B12 binding domain
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
The deposited structure of PDB entry 2xij contains 1 copy of Pfam domain PF02310 (B12 binding domain) in Methylmalonyl-CoA mutase, mitochondrial. Showing 1 copy in chain A.

PF01642
Domain description: Methylmalonyl-CoA mutase
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
The deposited structure of PDB entry 2xij contains 1 copy of Pfam domain PF01642 (Methylmalonyl-CoA mutase) in Methylmalonyl-CoA mutase, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR006158
Domain description: Cobalamin (vitamin B12)-binding domain
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
IPR006099
Domain description: Methylmalonyl-CoA mutase, alpha/beta chain, catalytic
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
IPR006159
Domain description: Methylmalonyl-CoA mutase, C-terminal
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
IPR036724
Domain description: Cobalamin-binding domain superfamily
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
IPR006098
Domain description: Methylmalonyl-CoA mutase, alpha chain, catalytic
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
IPR016176
Domain description: Cobalamin (vitamin B12)-dependent enzyme, catalytic
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial

Structure domains

CATH CATH domains
3.40.50.280
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: Rossmann fold
Homology: Cobalamin-binding domain
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
The deposited structure of PDB entry 2xij contains 1 copy of CATH domain 3.40.50.280 (Rossmann fold) in Methylmalonyl-CoA mutase, mitochondrial. Showing 1 copy in chain A.
3.20.20.240
Class: Alpha Beta
Architecture: Alpha-Beta Barrel
Topology: TIM Barrel
Homology: Methylmalonyl-CoA mutase
Occurring in:
  1. Methylmalonyl-CoA mutase, mitochondrial
The deposited structure of PDB entry 2xij contains 1 copy of CATH domain 3.20.20.240 (TIM Barrel) in Methylmalonyl-CoA mutase, mitochondrial. Showing 1 copy in chain A.

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