Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
3.1.2.-
Hydrolases;
Acting on ester bonds;
Thioester hydrolases;
7.6.2.-
Translocases;
Catalysing the translocation of other compounds;
Linked to the hydrolysis of a nucleoside triphosphate;
| PDB | Resolution (Å) | PDB name |
|---|
| GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0015721 | bile acid and bile salt transport |
| Biological Process | GO:0006699 | bile acid biosynthetic process |
| Biological Process | GO:0006635 | fatty acid beta-oxidation |
| Biological Process | GO:0006633 | fatty acid biosynthetic process |
| Biological Process | GO:0015910 | long-chain fatty acid import into peroxisome |
| Biological Process | GO:0007031 | peroxisome organization |
| Biological Process | GO:1903512 | phytanic acid metabolic process |
| Biological Process | GO:0014070 | response to organic cyclic compound |
| Biological Process | GO:0009410 | response to xenobiotic stimulus |
| Biological Process | GO:0042760 | very long-chain fatty acid catabolic process |
| Biological Process | GO:0000038 | very long-chain fatty acid metabolic process |
| Molecular Function | GO:0140359 | ABC-type transporter activity |
| Molecular Function | GO:0005524 | ATP binding |
| Molecular Function | GO:0016887 | ATP hydrolysis activity |
| Molecular Function | GO:0042626 | ATPase-coupled transmembrane transporter activity |
| Molecular Function | GO:0047617 | acyl-CoA hydrolase activity |
| Molecular Function | GO:0005324 | long-chain fatty acid transporter activity |
| Molecular Function | GO:0042803 | protein homodimerization activity |
| Molecular Function | GO:0043621 | protein self-association |
| Cellular Component | GO:0005829 | cytosol |
| Cellular Component | GO:0043231 | intracellular membrane-bounded organelle |
| Cellular Component | GO:0016020 | membrane |
| Cellular Component | GO:0005739 | mitochondrion |
| Cellular Component | GO:0005782 | peroxisomal matrix |
| Cellular Component | GO:0005778 | peroxisomal membrane |
| Cellular Component | GO:0005777 | peroxisome |
| InterPro
|
InterPro name |
|---|---|
| IPR003439 | ABC transporter-like, ATP-binding domain |
| IPR003593 | AAA+ ATPase domain |
| IPR005283 | Peroxysomal long chain fatty acyl transporter |
| IPR011527 | ABC transporter type 1, transmembrane domain |
| IPR017871 | ABC transporter-like, conserved site |
| IPR027417 | P-loop containing nucleoside triphosphate hydrolase |
| IPR036640 | ABC transporter type 1, transmembrane domain superfamily |
| Pfam
|
Pfam name |
|---|---|
| PF00005 | ABC transporter |
| PF06472 | ABC transporter transmembrane region 2 |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-1369062 | ABC transporters in lipid homeostasis | Leaf | R-HSA-382551 | Transport of small molecules |
| R-HSA-8980692 | RHOA GTPase cycle | Leaf | R-HSA-162582 | Signal Transduction |
| R-HSA-9013106 | RHOC GTPase cycle | Leaf | R-HSA-162582 | Signal Transduction |
| R-HSA-9603798 | Class I peroxisomal membrane protein import | Leaf | R-HSA-9609507 | Protein localization |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Peroxisome membrane | ECO:0000269 | PubMed:10704444 |
| Peroxisome membrane | ECO:0000269 | PubMed:16344115 |
| Peroxisome membrane | ECO:0000269 | PubMed:17761678 |
| Peroxisome membrane | ECO:0000269 | PubMed:24333844 |
| Peroxisome membrane | ECO:0000269 | PubMed:29397936 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000007 | Autosomal recessive inheritance |
| HP:0000952 | Jaundice |
| HP:0001395 | Hepatic fibrosis |
| HP:0001399 | Hepatic failure |
| HP:0001409 | Portal hypertension |
| HP:0001744 | Splenomegaly |
| HP:0001891 | Iron deficiency anemia |
| HP:0002240 | Hepatomegaly |
| HP:0002904 | Hyperbilirubinemia |
| HP:0002910 | Elevated hepatic transaminase |
| HP:0003593 | Infantile onset |
| HP:0003645 | Prolonged partial thromboplastin time |
| HP:0003676 | Progressive |
| HP:0006580 | Portal fibrosis |
| HP:0012202 | Increased serum bile acid concentration |
| HP:0025196 | Increased total iron binding capacity |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| congenital bile acid synthesis defect 5 | MONDO:0014564 | K76 | chapter11, Diseases of the digestive system | OMIM:616278 |