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Details on Person UniProt:P03905 MT-ND4

Class:IdReferenceGeneProduct:60562
_chainChangeLogchain:1-459 added on Fri February 6 2015
_displayNameUniProt:P03905 MT-ND4
_timestamp2025-02-21 20:12:20
chainchain:1-459
checksum2EEE581F3BD270AF
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:15250827, PubMed:8344246, PubMed:8644732). Essential for the catalytic activity and assembly of complex I (PubMed:15250827, PubMed:8344246, PubMed:8644732).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.SUBCELLULAR LOCATION 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 complex I subunit 4 family.
descriptionrecommendedName: NADH-ubiquinone oxidoreductase chain 4 ecNumber evidence="6 12 13"7.1.1.2 alternativeName: NADH dehydrogenase subunit 4
geneNameMT-ND4
MTND4
NADH4
ND4
identifierP03905
isSequenceChangedFALSE
keyword3D-structure
Disease variant
Dystonia
Electron transport
Leber hereditary optic neuropathy
MELAS syndrome
Membrane
Mitochondrion
Mitochondrion inner membrane
NAD
Primary mitochondrial disease
Proteomics identification
Reference proteome
Respiratory chain
Translocase
Transmembrane
Transmembrane helix
Transport
Ubiquinone
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
nameMT-ND4
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998480] ENSEMBL:ENSG00000198886 MT-ND4 [Homo sapiens]
secondaryIdentifierNU4M_HUMAN
Q6RL39
Q6RQN9
Q8HNR8
sequenceLength459
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:60561] MT-ND4 [mitochondrial inner membrane] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P03905 MT-ND4 (60562)