_EC 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring). 30 PDB entries  
EC 1.-.-.- Oxidoreductases. [8,621 PDB entries]
EC 1.2.-.- Acting on the aldehyde or oxo group of donors. [495 PDB entries]
EC 1.2.4.- With a disulfide as acceptor. [66 PDB entries]
EC 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring). [30 PDB entries]    
1dtw

Pathway: Oxo-acid dehydrogenase complexes
Reaction: 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine- residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2).
 


3-methyl-2-oxobutanoate
+ [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
= [dihydrolipoyllysine- residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
+
CO(2)
Molecule diagrams generated from .mol files obtained from the KEGG ftp site.

Other 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.
Cofactor(s): Thiamine diphosphate.
 

Thiamine diphosphate
Molecule diagrams generated from .mol files obtained from the KEGG ftp site.
Comments: It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2- oxopentanoate and (S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of Ec 2.3.1.168, which also binds multiple copies of Ec 1.8.1.4. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in Ec 2.3.1.168.
Links:   [IntEnz]   [ExPASy]   [KEGG]  

There are 30 PDB entries in enzyme class E.C.1.2.4.4

  PDB code Protein
1dtw
Human branched-chain alpha-keto acid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B (382 residues) CATH domain: 3.40.50.970
Bound ligand:   Het Group TDP corresponds to enzyme cofactor Thiamine
1ols
Roles of his291-alpha and his146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B (386 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1olu
Roles of his291-alpha and his146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B (366 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1olx
Roles of his291-alpha and his146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B (391 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1qs0
Crystal structure of pseudomonas putida 2-oxoisovalerate deh (branched-chain alpha-keto acid dehydrogenase, e1b)
Source: Pseudomonas putida. Organism_taxid: 303. Expressed in: pseudomonas putida. Expression_system_taxid: 303.
Chains: A, B (407 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group COI is 60.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1u5b
Crystal structure of the human mitochondrial branched-chain ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Gene: bckdha. Expressed in: escherichia coli bl21. Expression_system_taxid: 511693. Gene: bckdhb.
Chains: A, B (391 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1um9
Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 in apo-form
Source: Thermus thermophilus. Organism_taxid: 274. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B, C, D (331 residues) CATH domain: 3.40.50.970
1umb
Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 in holo-form
Source: Thermus thermophilus. Organism_taxid: 274. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B, C, D (362 residues) CATH domain: 3.40.50.970
Bound ligand:   Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1umc
Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 with 4-methylpentanoate
Source: Thermus thermophilus. Organism_taxid: 274. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B, C, D (362 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group 4MV is 60.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1umd
Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 with 4-methyl-2-oxopentanoate as an intermediate
Source: Thermus thermophilus. Organism_taxid: 274. Expressed in: escherichia coli. Expression_system_taxid: 562.
Chains: A, B, C, D (362 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group COI is 75.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1v11
Crosstalk between cofactor binding and the phosphorylation loop conformation in the bckd machine
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Other_details: expression system used bl-21 cells with overexpressing groel and groes. Overexpressing groel and groes
Chains: A, B (370 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group BEN is 41.67% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1v16
Crosstalk between cofactor binding and the phosphorylation loop conformation in the bckd machine
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Other_details: expression system used bl-21 cells with overexpressing groel and groes. Overexpressing groel and groes
Chains: A, B (371 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group BEN is 41.67% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1v1m
Crosstalk between cofactor binding and the phosphorylation loop conformation in the bckd machine
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Groel and groes. Terminally tagged). Terminally tagged)
Chains: A, B (372 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group BEN is 41.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine diphosphate
1v1r
Crosstalk between cofactor binding and the phosphorylation loop conformation in the bckd machine
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Groes. Other_details: expression system used bl-21 cells with overexpressing groel and groes
Chains: A, B (352 residues) CATH domain: 3.40.50.970
Bound ligand:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
1wci
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 469008. Other_details: bl-21 cells with overexpressing groel and groes. Groes
Chains: A, B (388 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group WWF is 81.00% similar to enzyme cofactor Thiamine diphosphate
1x7w
Crystal structure of the human mitochondrial branched-chain ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Gene: bckdha. Expressed in: escherichia coli bl21. Expression_system_taxid: 511693. Gene: bckdhb.
Chains: A, B (369 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1x7x
Crystal structure of the human mitochondrial branched-chain ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Gene: bckdha. Expressed in: escherichia coli bl21. Expression_system_taxid: 511693. Gene: bckdhb.
Chains: A, B (370 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1x7y
Crystal structure of the human mitochondrial branched-chain ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Gene: bckdha. Expressed in: escherichia coli bl21. Expression_system_taxid: 511693. Gene: bckdhb.
Chains: A, B (369 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1x7z
Crystal structure of the human mitochondrial branched-chain ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Gene: bckdha. Expressed in: escherichia coli bl21. Expression_system_taxid: 511693. Gene: bckdhb.
Chains: A, B (391 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
1x80
Crystal structure of the human mitochondrial branched-chain ketoacid dehydrogenase
Source: Homo sapiens. Human. Organism_taxid: 9606. Gene: bckdha. Expressed in: escherichia coli bl21. Expression_system_taxid: 511693. Gene: bckdhb.
Chains: A, B (369 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
2beu
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Groes. Synthetic: yes
Chains: A, B (390 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group THV is 83.87% similar to enzyme cofactor Thiamine
2bev
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 511693. Synthetic: yes
Chains: A, B (390 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group THY is 81.25% similar to enzyme cofactor Thiamine
2bew
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 511693. Synthetic: yes
Chains: A, B (390 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group THW is 76.47% similar to enzyme cofactor Thiamine
2bfb
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Groes.
Chains: A, B (370 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group MRD is 60.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
2bfc
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Groes.
Chains: A, B (371 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TZD is 96.30% similar to enzyme cofactor Thiamine
2bfd
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 562. Groes.
Chains: A, B (374 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group MPD is 60.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
2bfe
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 511693. Other_details: cells with overexpressing groel and groes. Other_details: cells with overexpressing groel and groes
Chains: A, B (375 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group MRD is 60.00% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TDP corresponds to enzyme cofactor Thiamine
2bff
Reactivity modulation of human branched-chain alpha- ketoacid dehydrogenase by an internal molecular switch
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 511693. Other_details: expression strain overexpressing groel and g
Chains: A, B (392 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group TZD is 96.30% similar to enzyme cofactor Thiamine
2bp7
New crystal form of the pseudomonas putida branched-chain dehydrogenase (e1)
Source: Pseudomonas putida. Organism_taxid: 303. Expressed in: escherichia coli. Expression_system_taxid: 469008.
Chains: A, B, C, D, E, F, G, H (407 residues) CATH domain: 3.40.50.970
2j9f
Human branched-chain alpha-ketoacid dehydrogenase- decarboxylase e1b
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli. Expression_system_taxid: 51169. Expression_system_taxid: 511693.
Chains: A, B, C, D (383 residues) CATH domain: 3.40.50.970
Bound ligands:   Het Group GOL is 55.56% similar to enzyme reactant 3-methyl-2-oxobutanoate
  Het Group THV is 83.87% similar to enzyme cofactor Thiamine