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Details on Person UniProt:O75489 NDUFS3

Class:IdReferenceGeneProduct:60604
_chainChangeLogtransit peptide:1-36 added on Sat February 7 2015;chain:37-264 added on Sat February 7 2015
_displayNameUniProt:O75489 NDUFS3
_timestamp2025-02-21 20:11:58
chaintransit peptide:1-36
chain:37-264
checksumC058D62779BEF17B
commentFUNCTION Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:14729820, PubMed:30140060). Essential for the catalytic activity and assembly of complex I (PubMed:14729820, PubMed:24028823, PubMed:30140060).CATALYTIC ACTIVITY a ubiquinone + NADH + 5 H(+)(in) = a ubiquinol + NAD(+) + 4 H(+)(out)SUBUNIT Core subunit of respiratory chain NADH dehydrogenase (Complex I) which is composed of 45 different subunits (PubMed:12611891). Interacts with NDUFAF3 (PubMed:19463981). Interacts with RAB5IF (PubMed:31536960). Found in subcomplexes containing subunits NDUFS2, MT-ND1 and NDUFA13 (PubMed:17209039, PubMed:18826940).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the complex I 30 kDa subunit family.
descriptionrecommendedName: NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial ecNumber evidence="2 9"7.1.1.2 alternativeName: Complex I-30kD shortName: CI-30kD alternativeName: NADH-ubiquinone oxidoreductase 30 kDa subunit
geneNameNDUFS3
identifierO75489
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Direct protein sequencing
Disease variant
Electron transport
Membrane
Mitochondrion
Mitochondrion inner membrane
NAD
Oxidoreductase
Primary mitochondrial disease
Proteomics identification
Reference proteome
Respiratory chain
Transit peptide
Translocase
Transport
Ubiquinone
modified[InstanceEdit:84067] Schmidt, EE, 2003-12-18 04:29:09
[InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10
[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
[InstanceEdit:354386] Schmidt, EE, 2008-06-18 04:45:12
[InstanceEdit:384350] Kanapin, AA, 2008-11-26 14:00:39
[InstanceEdit:392885] Kanapin, AA, 2009-03-09 12:07:18
[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
[InstanceEdit:423310] Kanapin, AA
[InstanceEdit:435478] Kanapin, AA
[InstanceEdit:435871] Kanapin, AA
[InstanceEdit:447347] Kanapin, AA
[InstanceEdit:525883] Kanapin, AA
[InstanceEdit:613449] Kanapin, AA
[InstanceEdit:797602] Kanapin, AA
[InstanceEdit:937368] Yung, CK
[InstanceEdit:1042053] Yung, CK
[InstanceEdit:1220657] Yung, CK
[InstanceEdit:1300696] Yung, CK
[InstanceEdit:1301627] Yung, CK
[InstanceEdit:1551960] Weiser, JD
[InstanceEdit:1995863] Weiser, JD
[InstanceEdit:2132304] Weiser, JD
[InstanceEdit:2265580] Weiser, JD
[InstanceEdit:4341137] Weiser, JD
[InstanceEdit:5433710] Weiser, JD
[InstanceEdit:5618415] Weiser, JD
[InstanceEdit:5634237] Weiser, JD
[InstanceEdit:5673015] Weiser, JD
[InstanceEdit:8987656] Weiser, JD
[InstanceEdit:9027688] Weiser, JD
[InstanceEdit:9037114] Weiser, JD
[InstanceEdit:9637257] Weiser, JD
[InstanceEdit:9645038] Weiser, JD
[InstanceEdit:9657908] Weiser, JD
[InstanceEdit:9676415] Weiser, JD
[InstanceEdit:9688885] Weiser, JD
[InstanceEdit:9698430] Weiser, JD
[InstanceEdit:9706439] Weiser, JD
[InstanceEdit:9715482] Weiser, JD
[InstanceEdit:9730071] Weiser, JD
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameNDUFS3
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8997850] ENSEMBL:ENSG00000213619 NDUFS3 [Homo sapiens]
secondaryIdentifierNDUS3_HUMAN
B2R9J1
B4DFM8
Q9UNQ8
sequenceLength264
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8970637] UniProt:O75489-1 NDUFS3 [Homo sapiens]
[ReferenceIsoform:8970638] UniProt:O75489-2 NDUFS3 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:163927] NDUFS3 [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:9714751] NDUFS3 [cytosol] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:O75489 NDUFS3 (60604)