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Details on Person UniProt:P31040 SDHA
| Class:Id | ReferenceGeneProduct:53638 |
|---|---|
| _chainChangeLog | transit peptide:1-43 added on Sat February 7 2015;chain:44-664 added on Sat February 7 2015;transit peptide:1-43 removed on Fri May 6 2016;chain:44-664 removed on Fri May 6 2016;transit peptide:1-42 added on Fri May 6 2016;chain:43-664 added on Fri May 6 2016 |
| _displayName | UniProt:P31040 SDHA |
| _timestamp | 2025-02-21 19:53:53 |
| chain | transit peptide:1-42 chain:43-664 |
| checksum | 180B664E3FFD0B34 |
| comment | FUNCTION Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:10746566, PubMed:24781757). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity). Can act as a tumor suppressor (PubMed:20484225).CATALYTIC ACTIVITY a ubiquinone + succinate = a ubiquinol + fumarateCATALYTIC ACTIVITY (R)-malate + a quinone = enol-oxaloacetate + a quinolCATALYTIC ACTIVITY (S)-malate + a quinone = enol-oxaloacetate + a quinolCOFACTOR Enol-oxaloacetate inhibits the succinate dehydrogenase activity.PATHWAY Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1.SUBUNIT Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD (PubMed:37098072). Interacts with SDHAF2/SDH5; interaction is required for FAD attachment (PubMed:19628817, PubMed:32887801). Interacts with TRAP1 (PubMed:23747254). Interacts with LACC1 (PubMed:28593945).INTERACTION Phosphorylation at Tyr-215 is important for efficient electron transfer in complex II and the prevention of ROS generation.PTM Acetylated. Deacetylated by SIRT3.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily.SEQUENCE CAUTION Extended N-terminus.SEQUENCE CAUTION Differs extensively from that shown. |
| description | recommendedName: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial ecNumber evidence="5 28"1.3.5.1 alternativeName: Flavoprotein subunit of complex II shortName: Fp alternativeName: Malate dehydrogenase [quinone] flavoprotein subunit ecNumber evidence="1"1.1.5.- |
| geneName | SDHA SDH2 SDHF |
| identifier | P31040 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Alternative splicing Cardiomyopathy Direct protein sequencing Disease variant Electron transport FAD Flavoprotein Leigh syndrome Membrane Mitochondrion Mitochondrion inner membrane Neurodegeneration Oxidoreductase Phosphoprotein Primary mitochondrial disease Proteomics identification Reference proteome Transit peptide Transport Tricarboxylic acid cycle Tumor suppressor |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 |
| name | SDHA |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:9003812] ENSEMBL:ENSG00000073578 SDHA [Homo sapiens] |
| secondaryIdentifier | SDHA_HUMAN A8K5J6 B4DJ60 E9PBJ5 Q16395 Q59GW8 Q8IW48 Q9UMY5 |
| sequenceLength | 664 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:8968527] UniProt:P31040-1 SDHA [Homo sapiens] [ReferenceIsoform:8968528] UniProt:P31040-2 SDHA [Homo sapiens] [ReferenceIsoform:8968529] UniProt:P31040-3 SDHA [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:70983] FAD-SDHA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9854690] SDHA [mitochondrial matrix] [Homo sapiens] |
| (referenceSequence) | [ModifiedResidue:9853508] 1'-(8alpha-FAD)-L-histidine at 99 |
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No pathways have been reviewed or authored by UniProt:P31040 SDHA (53638)
