Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
2.3.1.155
Transferases;
Acyltransferases;
Transferring groups other than aminoacyl groups;
acetyl-CoA C-myristoyltransferase
2.3.1.16
Transferases;
Acyltransferases;
Transferring groups other than aminoacyl groups;
acetyl-CoA C-acyltransferase
| GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0071222 | cellular response to lipopolysaccharide |
| Biological Process | GO:0006635 | fatty acid beta-oxidation |
| Biological Process | GO:0010467 | gene expression |
| Molecular Function | GO:0003857 | 3-hydroxyacyl-CoA dehydrogenase activity |
| Molecular Function | GO:0003723 | RNA binding |
| Molecular Function | GO:0003985 | acetyl-CoA C-acetyltransferase activity |
| Molecular Function | GO:0003988 | acetyl-CoA C-acyltransferase activity |
| Molecular Function | GO:0050633 | acetyl-CoA C-myristoyltransferase activity |
| Molecular Function | GO:0004300 | enoyl-CoA hydratase activity |
| Molecular Function | GO:0106222 | lncRNA binding |
| Cellular Component | GO:0005783 | endoplasmic reticulum |
| Cellular Component | GO:0005740 | mitochondrial envelope |
| Cellular Component | GO:0016507 | mitochondrial fatty acid beta-oxidation multienzyme complex |
| Cellular Component | GO:0005743 | mitochondrial inner membrane |
| Cellular Component | GO:0042645 | mitochondrial nucleoid |
| Cellular Component | GO:0005741 | mitochondrial outer membrane |
| Cellular Component | GO:0005739 | mitochondrion |
| InterPro
|
InterPro name |
|---|---|
| IPR002155 | Thiolase |
| IPR016039 | Thiolase-like |
| IPR020610 | Thiolase, active site |
| IPR020613 | Thiolase, conserved site |
| IPR020615 | Thiolase, acyl-enzyme intermediate active site |
| IPR020616 | Thiolase, N-terminal |
| IPR020617 | Thiolase, C-terminal |
| Pfam
|
Pfam name |
|---|---|
| PF00108 | Thiolase, N-terminal domain |
| PF02803 | Thiolase, C-terminal domain |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-1482798 | Acyl chain remodeling of CL | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77285 | Beta oxidation of myristoyl-CoA to lauroyl-CoA | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77288 | mitochondrial fatty acid beta-oxidation of unsaturated fatty acids | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77305 | Beta oxidation of palmitoyl-CoA to myristoyl-CoA | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77310 | Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77346 | Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77348 | Beta oxidation of octanoyl-CoA to hexanoyl-CoA | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-77350 | Beta oxidation of hexanoyl-CoA to butanoyl-CoA | Leaf | R-HSA-1430728 | Metabolism |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Endoplasmic reticulum | ECO:0000269 | PubMed:21527675 |
| Mitochondrion | ECO:0000269 | PubMed:21527675 |
| Mitochondrion inner membrane | ECO:0000269 | PubMed:21527675 |
| Mitochondrion outer membrane | ECO:0000269 | PubMed:21527675 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000580 | Pigmentary retinopathy |
| HP:0000829 | Hypoparathyroidism |
| HP:0001250 | Seizure |
| HP:0001252 | Hypotonia |
| HP:0001254 | Lethargy |
| HP:0001259 | Coma |
| HP:0001270 | Motor delay |
| HP:0001284 | Areflexia |
| HP:0001324 | Muscle weakness |
| HP:0001396 | Cholestasis |
| HP:0001531 | Failure to thrive in infancy |
| HP:0001635 | Congestive heart failure |
| HP:0001638 | Cardiomyopathy |
| HP:0001653 | Mitral regurgitation |
| HP:0001712 | Left ventricular hypertrophy |
| HP:0001761 | Pes cavus |
| HP:0001985 | Hypoketotic hypoglycemia |
| HP:0002033 | Poor suck |
| HP:0002093 | Respiratory insufficiency |
| HP:0002359 | Frequent falls |
| HP:0002476 | Primitive reflex |
| HP:0002878 | Respiratory failure |
| HP:0002901 | Hypocalcemia |
| HP:0003201 | Rhabdomyolysis |
| HP:0003324 | Generalized muscle weakness |
| HP:0003326 | Myalgia |
| HP:0003394 | Muscle spasm |
| HP:0003487 | Babinski sign |
| HP:0003546 | Exercise intolerance |
| HP:0003551 | Difficulty climbing stairs |
| HP:0003756 | Skeletal myopathy |
| HP:0005180 | Tricuspid regurgitation |
| HP:0006555 | Diffuse hepatic steatosis |
| HP:0007067 | Distal peripheral sensory neuropathy |
| HP:0007141 | Sensorimotor neuropathy |
| HP:0007340 | Lower limb muscle weakness |
| HP:0008110 | Equinovarus deformity |
| HP:0008138 | Equinus calcaneus |
| HP:0008872 | Feeding difficulties in infancy |
| HP:0009063 | Progressive distal muscle weakness |
| HP:0009830 | Peripheral neuropathy |
| HP:0011675 | Arrhythmia |
| HP:0011808 | Decreased patellar reflex |
| HP:0025145 | Rigors |
| HP:0030051 | Tip-toe gait |
| HP:0100626 | Chronic hepatic failure |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| mitochondrial trifunctional protein deficiency | MONDO:0012172 | G71 | chapter6, Diseases of the nervous system | OMIM:609015 | Orphanet:746 |