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Details on Person UniProt:P31040 SDHA

Class:IdReferenceGeneProduct:53638
_chainChangeLogtransit 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
_displayNameUniProt:P31040 SDHA
_timestamp2025-02-21 19:53:53
chaintransit peptide:1-42
chain:43-664
checksum180B664E3FFD0B34
commentFUNCTION 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.
descriptionrecommendedName: 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.-
geneNameSDHA
SDH2
SDHF
identifierP31040
isSequenceChangedFALSE
keyword3D-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
nameSDHA
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9003812] ENSEMBL:ENSG00000073578 SDHA [Homo sapiens]
secondaryIdentifierSDHA_HUMAN
A8K5J6
B4DJ60
E9PBJ5
Q16395
Q59GW8
Q8IW48
Q9UMY5
sequenceLength664
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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