ECHA_HUMAN
Evidence for PS localization
Functions
Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed, PubMed, PubMed, PubMed). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed, PubMed, PubMed). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed). It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed). [View more on UniProt]
Protein features
Binary interactions with known binding regions
To display all evidence describing the interaction with a partner, click on the protein name left to the bars.
Network
Isoforms
Disease-causing germline mutations
No data found.
Linear motifs
No annotated instance was found. To search for linear motifs, use the ELM prediction server.
Fingerprint
Molecular function
Term | Name | % | Distance from the top of the tree | Annotated in ECHA_HUMAN |
---|---|---|---|---|
GO:0043168 | anion binding | 36.8715 | 4 | yes |
GO:0000166 | nucleotide binding | 36.3128 | 4 | yes |
GO:0017076 | purine nucleotide binding | 34.0782 | 5 | yes |
GO:0032555 | purine ribonucleotide binding | 32.4022 | 4 | yes |
GO:0035639 | purine ribonucleoside triphosphate binding | 31.2849 | 4 | no |
GO:0030554 | adenyl nucleotide binding | 27.3743 | 6 | yes |
GO:0043169 | cation binding | 27.3743 | 4 | no |
GO:0046872 | metal ion binding | 26.2570 | 5 | no |
GO:0032559 | adenyl ribonucleotide binding | 25.6983 | 5 | yes |
GO:0005524 | ATP binding | 24.5810 | 5 | no |
GO:0003723 | RNA binding | 24.0223 | 4 | no |
GO:0016747 | acyltransferase activity, transferring groups other than amino-acyl groups | 3.3520 | 4 | yes |
GO:0016616 | oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor | 2.7933 | 4 | yes |
GO:0016836 | hydro-lyase activity | 2.7933 | 4 | yes |
GO:0003857 | 3-hydroxyacyl-CoA dehydrogenase activity | 2.2346 | 5 | yes |
GO:0004300 | enoyl-CoA hydratase activity | 2.2346 | 5 | yes |
GO:0051287 | NAD binding | 2.2346 | 7 | yes |
GO:0003985 | acetyl-CoA C-acetyltransferase activity | 1.6760 | 7 | yes |
GO:0003988 | acetyl-CoA C-acyltransferase activity | 1.6760 | 6 | yes |
GO:0016407 | acetyltransferase activity | 1.6760 | 5 | yes |
GO:0016408 | C-acyltransferase activity | 1.6760 | 5 | yes |
GO:0016453 | C-acetyltransferase activity | 1.6760 | 6 | yes |
GO:0000062 | fatty-acyl-CoA binding | 1.1173 | 4 | yes |
GO:0016509 | long-chain-3-hydroxyacyl-CoA dehydrogenase activity | 1.1173 | 6 | yes |
GO:0018812 | 3-hydroxyacyl-CoA dehydratase activity | 1.1173 | 6 | yes |
Biological process
Term | Name | % | Distance from top the of the tree | Annotated in ECHA_HUMAN |
---|---|---|---|---|
GO:0048522 | positive regulation of cellular process | 50.2793 | 4 | no |
GO:0048523 | negative regulation of cellular process | 44.6927 | 4 | no |
GO:0060255 | regulation of macromolecule metabolic process | 44.6927 | 4 | no |
GO:0031323 | regulation of cellular metabolic process | 44.1341 | 4 | no |
GO:0080090 | regulation of primary metabolic process | 44.1341 | 4 | no |
GO:0010646 | regulation of cell communication | 41.3408 | 4 | no |
GO:0051128 | regulation of cellular component organization | 39.6648 | 4 | no |
GO:0006996 | organelle organization | 38.5475 | 4 | no |
GO:0009893 | positive regulation of metabolic process | 37.9888 | 4 | no |
GO:0022607 | cellular component assembly | 36.8715 | 4 | no |
GO:0009889 | regulation of biosynthetic process | 35.1955 | 4 | no |
GO:0009966 | regulation of signal transduction | 35.1955 | 4 | no |
GO:0051246 | regulation of protein metabolic process | 35.1955 | 5 | no |
GO:0031326 | regulation of cellular biosynthetic process | 34.6369 | 5 | no |
GO:0010604 | positive regulation of macromolecule metabolic process | 34.0782 | 5 | no |
GO:0010556 | regulation of macromolecule biosynthetic process | 32.9609 | 5 | no |
GO:0010468 | regulation of gene expression | 31.8436 | 6 | no |
GO:0031325 | positive regulation of cellular metabolic process | 31.2849 | 5 | no |
GO:0008104 | protein localization | 29.0503 | 4 | no |
GO:0006796 | phosphate-containing compound metabolic process | 28.4916 | 4 | yes |
GO:0009892 | negative regulation of metabolic process | 27.9330 | 4 | no |
GO:0042981 | regulation of apoptotic process | 26.8156 | 5 | no |
GO:0043067 | regulation of programmed cell death | 26.8156 | 4 | no |
GO:0048584 | positive regulation of response to stimulus | 26.2570 | 4 | no |
GO:1902531 | regulation of intracellular signal transduction | 26.2570 | 5 | no |
Disease
No data found.
All partners
Uniprot ID | Details | Highest evidence | Localizing into PSD | HPA (protein expression in neurons) |
---|---|---|---|---|
1433T_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
AAKB1_HUMAN | [view interactions] | High throughput | no | yes |
AAR2_HUMAN | [view interactions] | High throughput | no | no |
AASS_HUMAN | [view interactions] | High throughput | no | yes |
ABCF2_HUMAN | [view interactions] | High throughput | no | yes |
ACAD9_HUMAN | [view interactions] | High throughput | no | yes |
ACADL_HUMAN | [view interactions] | High throughput | no | no |
ACADM_HUMAN | [view interactions] | High throughput | no | yes |
ACADS_HUMAN | [view interactions] | High throughput | no | yes |
ACADV_HUMAN | [view interactions] | High throughput | no | yes |
ACD11_HUMAN | [view interactions] | High throughput | no | yes |
ACE2_HUMAN | [view interactions] | High throughput | no | no |
ACOT9_HUMAN | [view interactions] | High throughput | no | yes |
ACPM_HUMAN | [view interactions] | High throughput | no | yes |
ACTB_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
ACTC_HUMAN | [view interactions] | High throughput | no | no |
ADAS_HUMAN | [view interactions] | High throughput | no | yes |
ADNP_HUMAN | [view interactions] | High throughput | no | yes |
AGR2_HUMAN | [view interactions] | High throughput | no | no |
AGRIN_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
AIFM1_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
AKIP_HUMAN | [view interactions] | High throughput | no | yes |
AKTIP_HUMAN | [view interactions] | High throughput | no | yes |
ALK_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
ALKB3_HUMAN | [view interactions] | High throughput | no | yes |