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CROSSTALK BETWEEN COFACTOR BINDING AND THE PHOSPHORYLATION LOOP CONFORMATION IN THE BCKD MACHINE

Source organism: Homo sapiens
Biochemical function: catalytic activity
Biological process: branched-chain amino acid catabolic process
Cellular component: obsolete mitochondrial branched-chain alpha-keto acid dehydrogenase complex

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EC 1.2.4.4: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

Reaction catalysed:
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2)
Systematic name:
3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating)
Alternative Name(s):
  • 2-oxoisocaproate dehydrogenase
  • 2-oxoisovalerate (lipoate) dehydrogenase
  • 3-methyl-2-oxobutanoate dehydrogenase (lipoamide)
  • 3-methyl-2-oxobutanoate:lipoamide oxidoreductase (decarboxylating and acceptor-2-methylpropanoylating)
  • Alpha-keto-alpha-methylvalerate dehydrogenase
  • Alpha-ketoisocaproate dehydrogenase
  • Alpha-ketoisocaproic dehydrogenase
  • Alpha-ketoisocaproic-alpha-keto-alpha-methylvaleric dehydrogenase
  • Alpha-ketoisovalerate dehydrogenase
  • Alpha-oxoisocaproate dehydrogenase
  • BCKDH
  • BCOAD
  • Branched chain keto acid dehydrogenase
  • Branched-chain (-2-oxoacid) dehydrogenase (BCD)
  • Branched-chain 2-keto acid dehydrogenase
  • Branched-chain 2-oxo acid dehydrogenase
  • Branched-chain alpha-keto acid dehydrogenase
  • Branched-chain alpha-oxo acid dehydrogenase
  • Branched-chain keto acid dehydrogenase
  • Branched-chain ketoacid dehydrogenase
  • Dehydrogenase, 2-oxoisovalerate (lipoate)
  • Dehydrogenase, branched chain alpha-keto acid

GO terms

Sequence families

Pfam Protein families (Pfam)
PF02780
Domain description: Transketolase, C-terminal domain
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of Pfam domain PF02780 (Transketolase, C-terminal domain) in 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial. Showing 1 copy in chain B.

PF02779
Domain description: Transketolase, pyrimidine binding domain
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of Pfam domain PF02779 (Transketolase, pyrimidine binding domain) in 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial. Showing 1 copy in chain B.

PF00676
Domain description: Dehydrogenase E1 component
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of Pfam domain PF00676 (Dehydrogenase E1 component) in 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR009014
Domain description: Transketolase C-terminal/Pyruvate-ferredoxin oxidoreductase domain II
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
IPR005475
Domain description: Transketolase-like, pyrimidine-binding domain
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
IPR029061
Domain description: Thiamin diphosphate-binding fold
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
  2. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
IPR033248
Domain description: Transketolase, C-terminal domain
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
IPR001017
Domain description: Dehydrogenase, E1 component
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Structure domains

CATH CATH domains
3.40.50.970
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: Rossmann fold
Homology: Rossmann fold
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
  2. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of CATH domain 3.40.50.970 (Rossmann fold) in 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial. Showing 1 copy in chain A.
The deposited structure of PDB entry 1v11 contains 1 copy of CATH domain 3.40.50.970 (Rossmann fold) in 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial. Showing 1 copy in chain B.
3.40.50.920
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: Rossmann fold
Homology: Rossmann fold
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of CATH domain 3.40.50.920 (Rossmann fold) in 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial. Showing 1 copy in chain B.
SCOP SCOP annotations
52926
Class: Alpha and beta proteins (a/b)
Fold: TK C-terminal domain-like
Superfamily: TK C-terminal domain-like
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of SCOP domain 52926 (Branched-chain alpha-keto acid dehydrogenase beta-subunit, C-terminal-domain) in 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial. Showing 1 copy in chain B.
88741
Class: Alpha and beta proteins (a/b)
Fold: Thiamin diphosphate-binding fold (THDP-binding)
Superfamily: Thiamin diphosphate-binding fold (THDP-binding)
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of SCOP domain 88741 (Branched-chain alpha-keto acid dehydrogenase Pyr module) in 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial. Showing 1 copy in chain B.
88766
Class: Alpha and beta proteins (a/b)
Fold: Thiamin diphosphate-binding fold (THDP-binding)
Superfamily: Thiamin diphosphate-binding fold (THDP-binding)
Occurring in:
  1. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
The deposited structure of PDB entry 1v11 contains 1 copy of SCOP domain 88766 (Branched-chain alpha-keto acid dehydrogenase PP module) in 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial. Showing 1 copy in chain A.

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