HADHA — Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
HADHA belongs to a gene co-expression module in 4 of 28 SCUBA cell types. Each module groups genes that rise and fall together in that cell type; the genes it shares a module with are its closest co-expression partners there.
HADHA's module in each cell type
| Cell type | Module | Shares the module with | |
|---|---|---|---|
| Enterocytes | Oxidative Phosphorylation Mitochondrial & OxPhos | COX6A1, COX6C, DDT, ECHS1, LGALS4, MGST3, NDUFA1, PRDX1 +3 more | View in SCUBA |
| Gamma-delta T cells | DNA Damage Repair DNA/chromatin regulation | AGGF1, ANP32A, DCK, DDX46, HDDC2, HENMT1, IFNAR1, LYPLA1 +8 more | |
| Innate lymphoid cells | Septin Cytoskeleton Cytoskeleton & motility | ATP6V0C, C17orf49, CIAO2A, COX16, DARS1, DDOST, GET3, H2AC6 +18 more | View in SCUBA |
| Macrophages | RNA Processing & Repair Housekeeping | BRD7, CCDC59, CCT4, CCT6A, CNDP2, EIF3J, EIF5B, EMC4 +31 more | View in SCUBA |
About the gene
| Synonyms | GBP, LCEH, LCHAD, MTPA |
|---|---|
| Chromosome | 2: 26190635-26244672 |
| Predicted location | Intracellular |
| Essential gene | No |
| Protein class | Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets, Predicted intracellular proteins |
| Molecular function | Lyase, Multifunctional enzyme, Oxidoreductase, Transferase |
| Biological process | Fatty acid metabolism, Lipid metabolism |
Function
Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. 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. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. 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. Independently of subunit beta, HADHA also exhibits a cardiolipin acyltransferase activity that participates in cardiolipin remodeling; cardiolipin is a major mitochondrial membrane phospholipid. HADHA may act downstream of Tafazzin/TAZ, that remodels monolysocardiolipin (MLCL) to a cardiolipin intermediate, and then HADHA may continue to remodel this species into mature tetralinoleoyl-cardiolipin. Has also been proposed to act directly on MLCL; capable of acylating MLCL using different acyl-CoA substrates, with highest activity for oleoyl-CoA.
Human Protein Atlas · Open Targets · UniProt
Gene annotation from the Human Protein Atlas and UniProt; see sources & licences.