Details of Protein
| General Information of Protein (ID: PRT00733) | |||||
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| Name | Monolysocardiolipin acyltransferase (HADHA) | ||||
| Synonyms |
Click to Show/Hide Synonyms of This Protein
78 kDa gastrin-binding protein; Trifunctional enzyme subunit alpha, mitochondrial; TP-alpha; HADHA; HADH
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| Gene Name | HADHA | Gene ID | |||
| UniProt ID | |||||
| Family | Transferases (EC 2) | ||||
| EC Number | EC: 2.3.1.- (Click to Show/Hide the Complete EC Tree) | ||||
| Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein | |||||
| Sequence |
MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSK
VNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLS QEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALP GAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYL EEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPA PLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKD VKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSF ERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALP ISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVK DGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVA EDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLK LPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRF VDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ |
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| Structure | |||||
| 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. | ||||
| Regulatory Network | |||||
| Full List of Metabolite(s) Regulating This Protein | ||||||
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| Organic acids and derivatives | ||||||
| Leucine | Click to Show/Hide the Full List of Regulating Pair(s): 1 Pair(s) | |||||
| Detailed Information |
Metabo Info
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| Regulating Pair |
Experim Info
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[1] | ||||
| Introduced Variation | Leucine addition (5 hours) | |||||
| Induced Change | HADHA protein expression levels: decrease | |||||
| Summary | Introduced Variation
Induced Change
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| Disease Status | Healthy individual | |||||
| Details | It is reported that leucine addition causes the decrease of HADHA protein expression compared with control group. | |||||
| References | |||||
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| 1 | An enteral leucine supply modulates human duodenal mucosal proteome and decreases the expression of enzymes involved in fatty acid beta-oxidation. J Proteomics. 2013 Jan 14;78:535-44. | ||||
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