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Crystal structure of Cys315Ala mutant of human mitochondrial branched chain aminotransferase

Source organism: Homo sapiens
Biochemical function: L-isoleucine-2-oxoglutarate transaminase activity
Biological process: cellular response to leukemia inhibitory factor
Cellular component: mitochondrial matrix

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EC 2.6.1.42: Branched-chain-amino-acid transaminase

Reaction catalysed:
L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate
Systematic name:
Branched-chain-amino-acid:2-oxoglutarate aminotransferase
Alternative Name(s):
  • Branched-chain amino acid aminotransferase
  • Branched-chain amino acid--glutamate transaminase
  • Branched-chain aminotransferase
  • Glutamate--branched-chain amino acid transaminase
  • L-branched chain amino acid aminotransferase
  • Transaminase B

GO terms

Biochemical function:
Biological process:
Cellular component:

Sequence family

Pfam Protein family (Pfam)
PF01063
Domain description: Amino-transferase class IV
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
The deposited structure of PDB entry 2hgx contains 2 copies of Pfam domain PF01063 (Amino-transferase class IV) in Branched-chain-amino-acid aminotransferase, mitochondrial. Showing 1 copy in chain B.

InterPro InterPro annotations
IPR005786
Domain description: Branched-chain amino acid aminotransferase II
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
IPR018300
Domain description: Aminotransferase, class IV, conserved site
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
IPR043132
Domain description: Branched-chain-amino-acid aminotransferase-like, C-terminal
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
IPR001544
Domain description: Aminotransferase class IV
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
IPR033939
Domain description: Branched-chain aminotransferase
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
IPR036038
Domain description: Aminotransferase-like, PLP-dependent enzymes
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
IPR043131
Domain description: Branched-chain-amino-acid aminotransferase-like, N-terminal
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial

Structure domains

CATH CATH domains
3.30.470.10
Class: Alpha Beta
Architecture: 2-Layer Sandwich
Topology: D-amino Acid Aminotransferase; Chain A, domain 1
Homology: D-amino Acid Aminotransferase; Chain A, domain 1
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
The deposited structure of PDB entry 2hgx contains 2 copies of CATH domain 3.30.470.10 (D-amino Acid Aminotransferase; Chain A, domain 1) in Branched-chain-amino-acid aminotransferase, mitochondrial. Showing 1 copy in chain B.
3.20.10.10
Class: Alpha Beta
Architecture: Alpha-Beta Barrel
Topology: D-amino Acid Aminotransferase; Chain A, domain 2
Homology: D-amino Acid Aminotransferase, subunit A, domain 2
Occurring in:
  1. Branched-chain-amino-acid aminotransferase, mitochondrial
The deposited structure of PDB entry 2hgx contains 2 copies of CATH domain 3.20.10.10 (D-amino Acid Aminotransferase; Chain A, domain 2) in Branched-chain-amino-acid aminotransferase, mitochondrial. Showing 1 copy in chain B.

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