| 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 the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity. 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. Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity. |
| Protein Name | Trifunctional Enzyme Subunit Alpha - Mitochondrial78 Kda Gastrin-Binding ProteinMonolysocardiolipin AcyltransferaseTp-Alpha Includes - Long-Chain Enoyl-Coa Hydratase - Long Chain 3-Hydroxyacyl-Coa Dehydrogenase |
| Database Links | Reactome: R-HSA-1482798Reactome: R-HSA-77285Reactome: R-HSA-77288Reactome: R-HSA-77305Reactome: R-HSA-77310Reactome: R-HSA-77346Reactome: R-HSA-77348Reactome: R-HSA-77350 |
| Cellular Localisation | MitochondrionMitochondrion Inner MembraneProtein Stability And Association With Mitochondrion Inner Membrane Do Not Require Hadhb |
| Alternative Antibody Names | Anti-Trifunctional Enzyme Subunit Alpha - Mitochondrial antibodyAnti-78 Kda Gastrin-Binding Protein antibodyAnti-Monolysocardiolipin Acyltransferase antibodyAnti-Tp-Alpha Includes - Long-Chain Enoyl-Coa Hydratase - Long Chain 3-Hydroxyacyl-Coa Dehydrogenase antibodyAnti-HADHA antibodyAnti-HADH antibody |
Information sourced from Uniprot.org
![Confocal immunofluorescence analysis of U2OS cells using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299) at dilution of 1:100. Blue: DAPI for nuclear staining.](https://cdn11.bigcommerce.com/s-zso2xnchw9/images/stencil/124x110/products/101009/388183/STJ27299_2__69064.1713157620.jpg?c=1 "Confocal immunofluorescence analysis of U2OS cells using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299) at dilution of 1:100. Blue: DAPI for nuclear staining.")
![Immunofluorescence analysis of U2OS cells using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299). Secondary antibody: Cy3 Goat Anti-Rabbit IgG (H+L) at 1:500 dilution. Blue: DAPI for nuclear staining.](https://cdn11.bigcommerce.com/s-zso2xnchw9/images/stencil/124x110/products/101009/388184/STJ27299_3__60546.1713157621.jpg?c=1 "Immunofluorescence analysis of U2OS cells using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299). Secondary antibody: Cy3 Goat Anti-Rabbit IgG (H+L) at 1:500 dilution. Blue: DAPI for nuclear staining.")
![Western blot analysis of lysates from wild type (WT) and HADHA knockout (KO) 293T cells, using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299) at 1:5000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (STJS000856) at 1:10000 dilution. Lysates/proteins: 25 Mu g per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 1s.](https://cdn11.bigcommerce.com/s-zso2xnchw9/images/stencil/124x110/products/101009/388185/STJ27299_4__80697.1713157621.jpg?c=1 "Western blot analysis of lysates from wild type (WT) and HADHA knockout (KO) 293T cells, using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299) at 1:5000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (STJS000856) at 1:10000 dilution. Lysates/proteins: 25 Mu g per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 1s.")
![Western blot analysis of lysates from K-562 cells, using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299) at 1:5000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (STJS000856) at 1:10000 dilution. Lysates/proteins: 25 Mu g per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 1s.](https://cdn11.bigcommerce.com/s-zso2xnchw9/images/stencil/124x110/products/101009/388186/STJ27299_5__34793.1713157622.jpg?c=1 "Western blot analysis of lysates from K-562 cells, using [KO Validated] HADHA Rabbit polyclonal antibody (STJ27299) at 1:5000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (STJS000856) at 1:10000 dilution. Lysates/proteins: 25 Mu g per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 1s.")
