› › Function and Biology

Crystal Structure of Human Mitochondrial Trifunctional Protein

Source organism: Homo sapiens
Biochemical function: transferase activity
Biological process: fatty acid beta-oxidation
Cellular component: mitochondrial nucleoid

Quick links

EC 2.3.1.16: Acetyl-CoA C-acyltransferase

Reaction catalysed:
(1a) [acetyl-CoA C-acyltransferase]-S-acyl-L-cyteine + acetyl-CoA = 3-oxoacyl-CoA + [acetyl-CoA C-acyltransferase]-L-cyteine
Systematic name:
Acyl-CoA:acetyl-CoA C-acyltransferase
Alternative Name(s):
  • 2-methylacetoacetyl-CoA thiolase
  • 3-ketoacyl CoA thiolase
  • 3-ketoacyl coenzyme A thiolase
  • 3-ketoacyl thiolase
  • 3-ketoacyl-CoA thiolase
  • 3-ketothiolase
  • 3-oxoacyl-CoA thiolase
  • 3-oxoacyl-coenzyme A thiolase
  • 6-oxoacyl-CoA thiolase
  • Acetoacetyl-CoA beta-ketothiolase
  • Acetyl-CoA acyltransferase
  • Beta-ketoacyl coenzyme A thiolase
  • Beta-ketoacyl-CoA thiolase
  • Beta-ketoadipyl coenzyme A thiolase
  • Beta-ketoadipyl-CoA thiolase
  • Beta-ketothiolase
  • KAT
  • Ketoacyl-CoA acyltransferase
  • Ketoacyl-coenzyme A thiolase
  • Long-chain 3-oxoacyl-CoA thiolase
  • Oxoacyl-coenzyme A thiolase
  • Pro-3-ketoacyl-CoA thiolase
  • Thiolase I

EC 4.2.1.17: Enoyl-CoA hydratase

Reaction catalysed:
(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H(2)O
Systematic name:
(3S)-3-hydroxyacyl-CoA hydro-lyase
Alternative Name(s):
  • 2-enoyl-CoA hydratase
  • 2-octenoyl coenzyme A hydrase
  • Acyl coenzyme A hydrase
  • Beta-hydroxyacid dehydrase
  • Beta-hydroxyacyl-CoA dehydrase
  • Crotonase
  • Crotonyl hydrase
  • D-3-hydroxyacyl-CoA dehydratase
  • ECH
  • Enol-CoA hydratase
  • Enoyl coenzyme A hydrase (D)
  • Enoyl coenzyme A hydrase (L)
  • Enoyl coenzyme A hydratase
  • Enoyl hydrase
  • Hydratase, enoyl coenzyme A
  • Short chain enoyl coenzyme A hydratase
  • Short-chain enoyl-CoA hydratase
  • Trans-2-enoyl-CoA hydratase
  • Unsaturated acyl-CoA hydratase

EC 1.1.1.211: Long-chain-3-hydroxyacyl-CoA dehydrogenase

Reaction catalysed:
A long-chain (S)-3-hydroxyacyl-CoA + NAD(+) = a long-chain 3-oxoacyl-CoA + NADH
Systematic name:
Long-chain-(S)-3-hydroxyacyl-CoA:NAD(+) oxidoreductase
Alternative Name(s):
  • 3-hydroxyacyl-CoA dehydrogenase
  • Beta-hydroxyacyl-CoA dehydrogenase
  • LCHAD
  • Long-chain 3-hydroxyacyl coenzyme A dehydrogenase

EC 2.3.1.155: Acetyl-CoA C-myristoyltransferase

Reaction catalysed:
Myristoyl-CoA + acetyl-CoA = 3-oxopalmitoyl-CoA + CoA
Systematic name:
Myristoyl-CoA:acetyl-CoA C-myristoyltransferase
Alternative Name(s):
  • 3-oxopalmitoyl-CoA hydrolase
  • 3-oxopalmitoyl-CoA-CoA acetyltransferase
  • Myristoyl-CoA C-acetyltransferase

GO terms

Sequence families

Pfam Protein families (Pfam)
PF00725
Domain description: 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
The deposited structure of PDB entry 6dv2 contains 12 copies of Pfam domain PF00725 (3-hydroxyacyl-CoA dehydrogenase, C-terminal domain) in Trifunctional enzyme subunit alpha, mitochondrial. Showing 2 copies in chain G.

PF02737
Domain description: 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
The deposited structure of PDB entry 6dv2 contains 6 copies of Pfam domain PF02737 (3-hydroxyacyl-CoA dehydrogenase, NAD binding domain) in Trifunctional enzyme subunit alpha, mitochondrial. Showing 1 copy in chain G.

PF00378
Domain description: Enoyl-CoA hydratase/isomerase
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
The deposited structure of PDB entry 6dv2 contains 6 copies of Pfam domain PF00378 (Enoyl-CoA hydratase/isomerase) in Trifunctional enzyme subunit alpha, mitochondrial. Showing 1 copy in chain G.

PF02803
Domain description: Thiolase, C-terminal domain
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
The deposited structure of PDB entry 6dv2 contains 6 copies of Pfam domain PF02803 (Thiolase, C-terminal domain) in Trifunctional enzyme subunit beta, mitochondrial. Showing 1 copy in chain A.

PF00108
Domain description: Thiolase, N-terminal domain
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
The deposited structure of PDB entry 6dv2 contains 6 copies of Pfam domain PF00108 (Thiolase, N-terminal domain) in Trifunctional enzyme subunit beta, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR016039
Domain description: Thiolase-like
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR006108
Domain description: 3-hydroxyacyl-CoA dehydrogenase, C-terminal
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR008927
Domain description: 6-phosphogluconate dehydrogenase-like, C-terminal domain superfamily
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR002155
Domain description: Thiolase
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR006180
Domain description: 3-hydroxyacyl-CoA dehydrogenase, conserved site
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR006176
Domain description: 3-hydroxyacyl-CoA dehydrogenase, NAD binding
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR036291
Domain description: NAD(P)-binding domain superfamily
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR018376
Domain description: Enoyl-CoA hydratase/isomerase, conserved site
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR001753
Domain description: Enoyl-CoA hydratase/isomerase
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR029045
Domain description: ClpP/crotonase-like domain superfamily
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR012803
Domain description: Fatty acid oxidation complex, alpha subunit, mitochondrial
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
IPR020610
Domain description: Thiolase, active site
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR020613
Domain description: Thiolase, conserved site
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR020617
Domain description: Thiolase, C-terminal
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR020615
Domain description: Thiolase, acyl-enzyme intermediate active site
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR020616
Domain description: Thiolase, N-terminal
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial
IPR038418
Domain description: 6-pyruvoyl tetrahydropterin synthase/QueD superfamily
Occurring in:
  1. Trifunctional enzyme subunit beta, mitochondrial

Structure domain

CATH CATH domain
3.40.50.720
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: Rossmann fold
Homology: NAD(P)-binding Rossmann-like Domain
Occurring in:
  1. Trifunctional enzyme subunit alpha, mitochondrial
The deposited structure of PDB entry 6dv2 contains 6 copies of CATH domain 3.40.50.720 (Rossmann fold) in Trifunctional enzyme subunit alpha, mitochondrial. Showing 1 copy in chain G.

Quick links