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Details on Person UniProt:O14521 SDHD

Class:IdReferenceGeneProduct:53642
_chainChangeLogtransit peptide:1-56 added on Fri February 6 2015;chain:57-159 added on Fri February 6 2015
_displayNameUniProt:O14521 SDHD
_timestamp2024-11-03 19:40:38
chaintransit peptide:1-56
chain:57-159
checksum6B1AA94831C8C3B6
commentFUNCTION Membrane-anchoring 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:10482792, PubMed:9533030). 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).PATHWAY Carbohydrate metabolism; tricarboxylic acid cycle.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.INTERACTION 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 CybS family.ONLINE INFORMATION SDHD entry
descriptionrecommendedName: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial shortName: CybS alternativeName: CII-4 alternativeName: Malate dehydrogenase [quinone] cytochrome b small subunit alternativeName: QPs3 alternativeName: Succinate dehydrogenase complex subunit D alternativeName: Succinate-ubiquinone oxidoreductase cytochrome b small subunit alternativeName: Succinate-ubiquinone reductase membrane anchor subunit
geneNameSDHD
SDH4
identifierO14521
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Disease variant
Electron transport
Heme
Iron
Membrane
Metal-binding
Mitochondrion
Mitochondrion inner membrane
Primary mitochondrial disease
Proteomics identification
Reference proteome
Transit peptide
Transmembrane
Transmembrane helix
Transport
Tricarboxylic acid cycle
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9909836] Weiser, Joel, 2024-05-14
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameSDHD
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9001715] ENSEMBL:ENSG00000204370 SDHD [Homo sapiens]
secondaryIdentifierDHSD_HUMAN
A6ND90
B3KQQ8
E9PIC0
E9PIG3
E9PQI9
Q53XW5
Q6IRW2
sequenceLength159
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8975962] UniProt:O14521-1 SDHD [Homo sapiens]
[ReferenceIsoform:8975963] UniProt:O14521-2 SDHD [Homo sapiens]
[ReferenceIsoform:8975964] UniProt:O14521-3 SDHD [Homo sapiens]
[ReferenceIsoform:8975965] UniProt:O14521-4 SDHD [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:70989] SDHD [mitochondrial inner membrane] [Homo sapiens]
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