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Protein

NADH-ubiquinone oxidoreductase chain 4

Gene

MT-ND4

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity).By similarity

Catalytic activityi

NADH + ubiquinone + 5 H+(In) = NAD+ + ubiquinol + 4 H+(Out).

GO - Molecular functioni

  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
  • ubiquinone binding Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionTranslocase
Biological processElectron transport, Respiratory chain, Transport
LigandNAD, Ubiquinone

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis

Names & Taxonomyi

Protein namesi
Recommended name:
NADH-ubiquinone oxidoreductase chain 4 (EC:7.1.1.2)
Alternative name(s):
NADH dehydrogenase subunit 4
Gene namesi
Name:MT-ND4
Synonyms:MTND4, NADH4, ND4
Encoded oniMitochondrion
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Mitochondrion

Organism-specific databases

EuPathDBiHostDB:ENSG00000198886.2
HGNCiHGNC:7459 MT-ND4
MIMi516003 gene
neXtProtiNX_P03905

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei22 – 42HelicalSequence analysisAdd BLAST21
Transmembranei60 – 80HelicalSequence analysisAdd BLAST21
Transmembranei102 – 122HelicalSequence analysisAdd BLAST21
Transmembranei145 – 165HelicalSequence analysisAdd BLAST21
Transmembranei196 – 216HelicalSequence analysisAdd BLAST21
Transmembranei224 – 244HelicalSequence analysisAdd BLAST21
Transmembranei257 – 277HelicalSequence analysisAdd BLAST21
Transmembranei284 – 303HelicalSequence analysisAdd BLAST20
Transmembranei308 – 330HelicalSequence analysisAdd BLAST23
Transmembranei351 – 371HelicalSequence analysisAdd BLAST21
Transmembranei391 – 411HelicalSequence analysisAdd BLAST21

Keywords - Cellular componenti

Membrane, Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Leber hereditary optic neuropathy (LHON)4 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
See also OMIM:535000
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_004760340R → H in LHON; primary mutation; almost no vision recovery; most frequent mutation; seems to have no effect on electron transfer activity of the complex in inner mitochondrial membrane preparations. 4 PublicationsCorresponds to variant dbSNP:rs199476112Ensembl.1
Leber hereditary optic neuropathy with dystonia (LDYT)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionPart of a spectrum of Leber hereditary optic neuropathy. It is characterized by the association of optic atrophy and central vision loss with dystonia.
See also OMIM:500001
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_008393313V → I in LDYT; possible rare primary mutation. 1 PublicationCorresponds to variant dbSNP:rs200873900Ensembl.1
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionGenetically heterogeneous disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.
See also OMIM:540000
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_004759109T → A in MELAS. 1 PublicationCorresponds to variant dbSNP:rs199476113Ensembl.1

Keywords - Diseasei

Disease mutation, Dystonia, Leber hereditary optic neuropathy, MELAS syndrome, Primary mitochondrial disease

Organism-specific databases

DisGeNETi4538
GeneReviewsiMT-ND4
MalaCardsiMT-ND4
MIMi500001 phenotype
535000 phenotype
540000 phenotype
OpenTargetsiENSG00000198886
Orphaneti104 Leber hereditary optic neuropathy
99718 Leber plus disease
255210 Maternally-inherited Leigh syndrome
550 MELAS
168609 Mitochondrial non-syndromic sensorineural deafness with susceptibility to aminoglycoside exposure
PharmGKBiPA31263

Chemistry databases

ChEMBLiCHEMBL4499
DrugBankiDB00157 NADH

Polymorphism and mutation databases

DMDMi128748

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001179421 – 459NADH-ubiquinone oxidoreductase chain 4Add BLAST459

Proteomic databases

EPDiP03905
PaxDbiP03905
PeptideAtlasiP03905
PRIDEiP03905
ProteomicsDBi51615

PTM databases

iPTMnetiP03905
PhosphoSitePlusiP03905

Expressioni

Gene expression databases

BgeeiENSG00000198886 Expressed in 203 organ(s), highest expression level in right adrenal gland
ExpressionAtlasiP03905 baseline and differential
GenevisibleiP03905 HS

Organism-specific databases

HPAiHPA053928

Interactioni

Protein-protein interaction databases

BioGridi110634, 31 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP03905
IntActiP03905, 15 interactors
MINTiP03905
STRINGi9606.ENSP00000354961

Chemistry databases

BindingDBiP03905

Structurei

3D structure databases

ProteinModelPortaliP03905
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I subunit 4 family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4845 Eukaryota
COG1008 LUCA
GeneTreeiENSGT00730000111316
HOGENOMiHOG000100683
HOVERGENiHBG093667
InParanoidiP03905
KOiK03881
OMAiITRWGNQ
OrthoDBiEOG091G0T7G
PhylomeDBiP03905
TreeFamiTF343520

Family and domain databases

InterProiView protein in InterPro
IPR000260 NADH4_N
IPR010227 NADH_Q_OxRdtase_chainM/4
IPR003918 NADH_UbQ_OxRdtase
IPR001750 ND/Mrp_mem
PANTHERiPTHR43507 PTHR43507, 1 hit
PfamiView protein in Pfam
PF01059 Oxidored_q5_N, 1 hit
PF00361 Proton_antipo_M, 1 hit
PRINTSiPR01437 NUOXDRDTASE4
TIGRFAMsiTIGR01972 NDH_I_M, 1 hit

Sequencei

Sequence statusi: Complete.

P03905-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MLKLIVPTIM LLPLTWLSKK HMIWINTTTH SLIISIIPLL FFNQINNNLF
60 70 80 90 100
SCSPTFSSDP LTTPLLMLTT WLLPLTIMAS QRHLSSEPLS RKKLYLSMLI
110 120 130 140 150
SLQISLIMTF TATELIMFYI FFETTLIPTL AIITRWGNQP ERLNAGTYFL
160 170 180 190 200
FYTLVGSLPL LIALIYTHNT LGSLNILLLT LTAQELSNSW ANNLMWLAYT
210 220 230 240 250
MAFMVKMPLY GLHLWLPKAH VEAPIAGSMV LAAVLLKLGG YGMMRLTLIL
260 270 280 290 300
NPLTKHMAYP FLVLSLWGMI MTSSICLRQT DLKSLIAYSS ISHMALVVTA
310 320 330 340 350
ILIQTPWSFT GAVILMIAHG LTSSLLFCLA NSNYERTHSR IMILSQGLQT
360 370 380 390 400
LLPLMAFWWL LASLANLALP PTINLLGELS VLVTTFSWSN ITLLLTGLNM
410 420 430 440 450
LVTALYSLYM FTTTQWGSLT HHINNMKPSF TRENTLMFMH LSPILLLSLN

PDIITGFSS
Length:459
Mass (Da):51,581
Last modified:July 21, 1986 - v1
Checksum:i2EEE581F3BD270AF
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti230V → L (PubMed:1377597).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_00859979A → P2 Publications1
Natural variantiVAR_004759109T → A in MELAS. 1 PublicationCorresponds to variant dbSNP:rs199476113Ensembl.1
Natural variantiVAR_008600109T → P1 Publication1
Natural variantiVAR_008601132I → T1 Publication1
Natural variantiVAR_008602294M → T1 Publication1
Natural variantiVAR_008393313V → I in LDYT; possible rare primary mutation. 1 PublicationCorresponds to variant dbSNP:rs200873900Ensembl.1
Natural variantiVAR_004760340R → H in LHON; primary mutation; almost no vision recovery; most frequent mutation; seems to have no effect on electron transfer activity of the complex in inner mitochondrial membrane preparations. 4 PublicationsCorresponds to variant dbSNP:rs199476112Ensembl.1
Natural variantiVAR_064565420T → A Detected in a patient with mitochondrial complex I deficiency; uncertain pathological significance. 1 PublicationCorresponds to variant dbSNP:rs879136236Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J01415 Genomic DNA Translation: AAB58952.1
V00662 Genomic DNA Translation: CAA24035.1
AY495090 Genomic DNA Translation: AAR92505.1
AY495091 Genomic DNA Translation: AAR92518.1
AY495092 Genomic DNA Translation: AAR92531.1
AY495093 Genomic DNA Translation: AAR92544.1
AY495094 Genomic DNA Translation: AAR92557.1
AY495095 Genomic DNA Translation: AAR92570.1
AY495096 Genomic DNA Translation: AAR92583.1
AY495097 Genomic DNA Translation: AAR92596.1
AY495098 Genomic DNA Translation: AAR92609.1
AY495099 Genomic DNA Translation: AAR92622.1
AY495100 Genomic DNA Translation: AAR92635.1
AY495101 Genomic DNA Translation: AAR92648.1
AY495102 Genomic DNA Translation: AAR92661.1
AY495103 Genomic DNA Translation: AAR92674.1
AY495104 Genomic DNA Translation: AAR92687.1
AY495106 Genomic DNA Translation: AAR92713.1
AY495107 Genomic DNA Translation: AAR92726.1
AY495108 Genomic DNA Translation: AAR92739.1
AY495109 Genomic DNA Translation: AAR92752.1
AY495110 Genomic DNA Translation: AAR92765.1
AY495111 Genomic DNA Translation: AAR92778.1
AY495112 Genomic DNA Translation: AAR92791.1
AY495113 Genomic DNA Translation: AAR92804.1
AY495114 Genomic DNA Translation: AAR92817.1
AY495115 Genomic DNA Translation: AAR92830.1
AY495116 Genomic DNA Translation: AAR92843.1
AY495117 Genomic DNA Translation: AAR92856.1
AY495118 Genomic DNA Translation: AAR92869.1
AY495119 Genomic DNA Translation: AAR92882.1
AY495120 Genomic DNA Translation: AAR92895.1
AY495121 Genomic DNA Translation: AAR92908.1
AY495122 Genomic DNA Translation: AAR92921.1
AY495123 Genomic DNA Translation: AAR92934.1
AY495124 Genomic DNA Translation: AAR92947.1
AY495125 Genomic DNA Translation: AAR92960.1
AY495126 Genomic DNA Translation: AAR92973.1
AY495127 Genomic DNA Translation: AAR92986.1
AY495128 Genomic DNA Translation: AAR92999.1
AY495129 Genomic DNA Translation: AAR93012.1
AY495130 Genomic DNA Translation: AAR93025.1
AY495131 Genomic DNA Translation: AAR93038.1
AY495132 Genomic DNA Translation: AAR93051.1
AY495133 Genomic DNA Translation: AAR93064.1
AY495134 Genomic DNA Translation: AAR93077.1
AY495135 Genomic DNA Translation: AAR93090.1
AY495136 Genomic DNA Translation: AAR93103.1
AY495137 Genomic DNA Translation: AAR93116.1
AY495138 Genomic DNA Translation: AAR93129.1
AY495139 Genomic DNA Translation: AAR93142.1
AY495140 Genomic DNA Translation: AAR93155.1
AY495141 Genomic DNA Translation: AAR93168.1
AY495142 Genomic DNA Translation: AAR93181.1
AY495143 Genomic DNA Translation: AAR93194.1
AY495144 Genomic DNA Translation: AAR93207.1
AY495145 Genomic DNA Translation: AAR93220.1
AY495146 Genomic DNA Translation: AAR93233.1
AY495147 Genomic DNA Translation: AAR93246.1
AY495148 Genomic DNA Translation: AAR93259.1
AY495149 Genomic DNA Translation: AAR93272.1
AY495150 Genomic DNA Translation: AAR93285.1
AY495151 Genomic DNA Translation: AAR93298.1
AY495152 Genomic DNA Translation: AAR93311.1
AY495153 Genomic DNA Translation: AAR93324.1
AY495154 Genomic DNA Translation: AAR93337.1
AY495155 Genomic DNA Translation: AAR93350.1
AY495156 Genomic DNA Translation: AAR93363.1
AY495157 Genomic DNA Translation: AAR93376.1
AY495158 Genomic DNA Translation: AAR93389.1
AY495159 Genomic DNA Translation: AAR93402.1
AY495160 Genomic DNA Translation: AAR93415.1
AY495161 Genomic DNA Translation: AAR93428.1
AY495162 Genomic DNA Translation: AAR93441.1
AY495163 Genomic DNA Translation: AAR93454.1
AY495164 Genomic DNA Translation: AAR93467.1
AY495165 Genomic DNA Translation: AAR93480.1
AY495167 Genomic DNA Translation: AAR93506.1
AY495168 Genomic DNA Translation: AAR93519.1
AY495169 Genomic DNA Translation: AAR93532.1
AY495171 Genomic DNA Translation: AAR93558.1
AY495172 Genomic DNA Translation: AAR93571.1
AY495173 Genomic DNA Translation: AAR93584.1
AY495174 Genomic DNA Translation: AAR93597.1
AY495175 Genomic DNA Translation: AAR93610.1
AY495176 Genomic DNA Translation: AAR93623.1
AY495177 Genomic DNA Translation: AAR93636.1
AY495178 Genomic DNA Translation: AAR93649.1
AY495179 Genomic DNA Translation: AAR93662.1
AY495180 Genomic DNA Translation: AAR93675.1
AY495181 Genomic DNA Translation: AAR93688.1
AY495182 Genomic DNA Translation: AAR93701.1
AY495183 Genomic DNA Translation: AAR93714.1
AY495184 Genomic DNA Translation: AAR93727.1
AY495185 Genomic DNA Translation: AAR93740.1
AY495186 Genomic DNA Translation: AAR93753.1
AY495187 Genomic DNA Translation: AAR93766.1
AY495188 Genomic DNA Translation: AAR93779.1
AY495189 Genomic DNA Translation: AAR93792.1
AY495190 Genomic DNA Translation: AAR93805.1
AY495191 Genomic DNA Translation: AAR93818.1
AY495192 Genomic DNA Translation: AAR93831.1
AY495193 Genomic DNA Translation: AAR93844.1
AY495194 Genomic DNA Translation: AAR93857.1
AY495239 Genomic DNA Translation: AAR94442.1
AY495240 Genomic DNA Translation: AAR94455.1
AY495241 Genomic DNA Translation: AAR94468.1
AY495242 Genomic DNA Translation: AAR94481.1
AY495244 Genomic DNA Translation: AAR94507.1
AY495246 Genomic DNA Translation: AAR94533.1
AY495247 Genomic DNA Translation: AAR94546.1
AY495248 Genomic DNA Translation: AAR94559.1
AY495249 Genomic DNA Translation: AAR94572.1
AY495252 Genomic DNA Translation: AAR94611.1
AY495267 Genomic DNA Translation: AAR94806.1
AY495268 Genomic DNA Translation: AAR94819.1
AY495269 Genomic DNA Translation: AAR94832.1
AY495270 Genomic DNA Translation: AAR94845.1
AY495271 Genomic DNA Translation: AAR94858.1
AY495272 Genomic DNA Translation: AAR94871.1
AY495273 Genomic DNA Translation: AAR94884.1
AY495274 Genomic DNA Translation: AAR94897.1
AY495275 Genomic DNA Translation: AAR94910.1
AY495276 Genomic DNA Translation: AAR94923.1
AY495277 Genomic DNA Translation: AAR94936.1
AY495278 Genomic DNA Translation: AAR94949.1
AY495279 Genomic DNA Translation: AAR94962.1
AY495280 Genomic DNA Translation: AAR94975.1
AY495281 Genomic DNA Translation: AAR94988.1
AY495282 Genomic DNA Translation: AAR95001.1
AY495283 Genomic DNA Translation: AAR95014.1
AY495284 Genomic DNA Translation: AAR95027.1
AY495285 Genomic DNA Translation: AAR95040.1
AY495286 Genomic DNA Translation: AAR95053.1
AY495287 Genomic DNA Translation: AAR95066.1
AY495288 Genomic DNA Translation: AAR95079.1
AY495289 Genomic DNA Translation: AAR95092.1
AY495290 Genomic DNA Translation: AAR95105.1
AY495291 Genomic DNA Translation: AAR95118.1
AY495292 Genomic DNA Translation: AAR95131.1
AY495293 Genomic DNA Translation: AAR95144.1
AY495294 Genomic DNA Translation: AAR95157.1
AY495295 Genomic DNA Translation: AAR95170.1
AY495297 Genomic DNA Translation: AAR95196.1
AY495298 Genomic DNA Translation: AAR95209.1
AY495299 Genomic DNA Translation: AAR95222.1
AY495300 Genomic DNA Translation: AAR95235.1
AY495301 Genomic DNA Translation: AAR95248.1
AY495302 Genomic DNA Translation: AAR95261.1
AY495303 Genomic DNA Translation: AAR95274.1
AY495304 Genomic DNA Translation: AAR95287.1
AY495305 Genomic DNA Translation: AAR95300.1
AY495306 Genomic DNA Translation: AAR95313.1
AY495307 Genomic DNA Translation: AAR95326.1
AY495308 Genomic DNA Translation: AAR95339.1
AY495309 Genomic DNA Translation: AAR95352.1
AY495310 Genomic DNA Translation: AAR95365.1
AY495311 Genomic DNA Translation: AAR95378.1
AY495312 Genomic DNA Translation: AAR95391.1
AY495313 Genomic DNA Translation: AAR95404.1
AY495314 Genomic DNA Translation: AAR95417.1
AY495315 Genomic DNA Translation: AAR95430.1
AY495316 Genomic DNA Translation: AAR95443.1
AY495317 Genomic DNA Translation: AAR95456.1
AY495318 Genomic DNA Translation: AAR95469.1
AY495319 Genomic DNA Translation: AAR95482.1
AY495320 Genomic DNA Translation: AAR95495.1
AY495321 Genomic DNA Translation: AAR95508.1
AY495322 Genomic DNA Translation: AAR95521.1
AY495323 Genomic DNA Translation: AAR95534.1
AY495324 Genomic DNA Translation: AAR95547.1
AY495325 Genomic DNA Translation: AAR95560.1
AY495326 Genomic DNA Translation: AAR95573.1
AY495327 Genomic DNA Translation: AAR95586.1
AY495328 Genomic DNA Translation: AAR95599.1
AY495329 Genomic DNA Translation: AAR95612.1
AY495330 Genomic DNA Translation: AAR95625.1
AF465942 Genomic DNA Translation: AAN14556.1
AF346963 Genomic DNA Translation: AAK17216.1
AF346964 Genomic DNA Translation: AAK17229.2
AF346966 Genomic DNA Translation: AAK17255.1
AF346967 Genomic DNA Translation: AAK17268.2
AF346968 Genomic DNA Translation: AAK17281.2
AF346969 Genomic DNA Translation: AAK17294.2
AF346971 Genomic DNA Translation: AAK17320.2
AF346974 Genomic DNA Translation: AAK17359.2
AF346975 Genomic DNA Translation: AAK17372.2
AF346977 Genomic DNA Translation: AAK17398.1
AF346978 Genomic DNA Translation: AAK17411.1
AF346980 Genomic DNA Translation: AAK17437.2
AF346981 Genomic DNA Translation: AAK17450.2
AF346982 Genomic DNA Translation: AAK17463.1
AF346985 Genomic DNA Translation: AAK17502.2
AF346986 Genomic DNA Translation: AAK17515.2
AF346987 Genomic DNA Translation: AAK17528.2
AF346989 Genomic DNA Translation: AAK17554.2
AF346990 Genomic DNA Translation: AAK17567.1
AF346993 Genomic DNA Translation: AAK17606.2
AF346994 Genomic DNA Translation: AAK17619.2
AF346996 Genomic DNA Translation: AAK17645.2
AF346997 Genomic DNA Translation: AAK17658.2
AF347000 Genomic DNA Translation: AAK17697.1
AF347002 Genomic DNA Translation: AAK17723.2
AF347003 Genomic DNA Translation: AAK17736.2
AF347004 Genomic DNA Translation: AAK17749.2
AF347005 Genomic DNA Translation: AAK17762.2
AF347006 Genomic DNA Translation: AAK17775.2
AF347007 Genomic DNA Translation: AAK17788.2
AF347010 Genomic DNA Translation: AAK17827.2
AF347011 Genomic DNA Translation: AAK17840.2
AF347012 Genomic DNA Translation: AAK17853.2
AF347013 Genomic DNA Translation: AAK17866.2
AF347014 Genomic DNA Translation: AAK17879.2
AF347015 Genomic DNA Translation: AAK17892.2
AY289051 Genomic DNA Translation: AAP47889.1
AY289052 Genomic DNA Translation: AAP47902.1
AY289055 Genomic DNA Translation: AAP47941.1
AY289056 Genomic DNA Translation: AAP47954.1
AY289058 Genomic DNA Translation: AAP47980.1
AY289059 Genomic DNA Translation: AAP47993.1
AY289060 Genomic DNA Translation: AAP48006.1
AY289061 Genomic DNA Translation: AAP48019.1
AY289062 Genomic DNA Translation: AAP48032.1
AY289063 Genomic DNA Translation: AAP48045.1
AY289065 Genomic DNA Translation: AAP48071.1
AY289068 Genomic DNA Translation: AAP48110.1
AY289069 Genomic DNA Translation: AAP48123.1
AY289070 Genomic DNA Translation: AAP48136.1
AY289071 Genomic DNA Translation: AAP48149.1
AY289073 Genomic DNA Translation: AAP48175.1
AY289074 Genomic DNA Translation: AAP48188.1
AY289075 Genomic DNA Translation: AAP48201.1
AY289076 Genomic DNA Translation: AAP48214.1
AY289077 Genomic DNA Translation: AAP48227.1
AY289079 Genomic DNA Translation: AAP48253.1
AY289080 Genomic DNA Translation: AAP48266.1
AY289081 Genomic DNA Translation: AAP48279.1
AY289082 Genomic DNA Translation: AAP48292.1
AY289083 Genomic DNA Translation: AAP48305.1
AY289084 Genomic DNA Translation: AAP48318.1
AY289085 Genomic DNA Translation: AAP48331.1
AY289087 Genomic DNA Translation: AAP48357.1
AY289088 Genomic DNA Translation: AAP48370.1
AY289090 Genomic DNA Translation: AAP48396.1
AY289091 Genomic DNA Translation: AAP48409.1
AY289092 Genomic DNA Translation: AAP48422.1
AY289093 Genomic DNA Translation: AAP48434.1
AY289094 Genomic DNA Translation: AAP48447.1
AY289095 Genomic DNA Translation: AAP48460.1
AY289096 Genomic DNA Translation: AAP48473.1
AY289097 Genomic DNA Translation: AAP48486.1
AY289098 Genomic DNA Translation: AAP48499.1
AY289099 Genomic DNA Translation: AAP48512.1
AY289100 Genomic DNA Translation: AAP48525.1
AY289101 Genomic DNA Translation: AAP48538.1
AY289102 Genomic DNA Translation: AAP48551.1
PIRiA00434 DNHUN4
RefSeqiYP_003024035.1, NC_012920.1

Genome annotation databases

EnsembliENST00000361381; ENSP00000354961; ENSG00000198886
GeneIDi4538
KEGGihsa:4538
UCSCiuc064xpj.1 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J01415 Genomic DNA Translation: AAB58952.1
V00662 Genomic DNA Translation: CAA24035.1
AY495090 Genomic DNA Translation: AAR92505.1
AY495091 Genomic DNA Translation: AAR92518.1
AY495092 Genomic DNA Translation: AAR92531.1
AY495093 Genomic DNA Translation: AAR92544.1
AY495094 Genomic DNA Translation: AAR92557.1
AY495095 Genomic DNA Translation: AAR92570.1
AY495096 Genomic DNA Translation: AAR92583.1
AY495097 Genomic DNA Translation: AAR92596.1
AY495098 Genomic DNA Translation: AAR92609.1
AY495099 Genomic DNA Translation: AAR92622.1
AY495100 Genomic DNA Translation: AAR92635.1
AY495101 Genomic DNA Translation: AAR92648.1
AY495102 Genomic DNA Translation: AAR92661.1
AY495103 Genomic DNA Translation: AAR92674.1
AY495104 Genomic DNA Translation: AAR92687.1
AY495106 Genomic DNA Translation: AAR92713.1
AY495107 Genomic DNA Translation: AAR92726.1
AY495108 Genomic DNA Translation: AAR92739.1
AY495109 Genomic DNA Translation: AAR92752.1
AY495110 Genomic DNA Translation: AAR92765.1
AY495111 Genomic DNA Translation: AAR92778.1
AY495112 Genomic DNA Translation: AAR92791.1
AY495113 Genomic DNA Translation: AAR92804.1
AY495114 Genomic DNA Translation: AAR92817.1
AY495115 Genomic DNA Translation: AAR92830.1
AY495116 Genomic DNA Translation: AAR92843.1
AY495117 Genomic DNA Translation: AAR92856.1
AY495118 Genomic DNA Translation: AAR92869.1
AY495119 Genomic DNA Translation: AAR92882.1
AY495120 Genomic DNA Translation: AAR92895.1
AY495121 Genomic DNA Translation: AAR92908.1
AY495122 Genomic DNA Translation: AAR92921.1
AY495123 Genomic DNA Translation: AAR92934.1
AY495124 Genomic DNA Translation: AAR92947.1
AY495125 Genomic DNA Translation: AAR92960.1
AY495126 Genomic DNA Translation: AAR92973.1
AY495127 Genomic DNA Translation: AAR92986.1
AY495128 Genomic DNA Translation: AAR92999.1
AY495129 Genomic DNA Translation: AAR93012.1
AY495130 Genomic DNA Translation: AAR93025.1
AY495131 Genomic DNA Translation: AAR93038.1
AY495132 Genomic DNA Translation: AAR93051.1
AY495133 Genomic DNA Translation: AAR93064.1
AY495134 Genomic DNA Translation: AAR93077.1
AY495135 Genomic DNA Translation: AAR93090.1
AY495136 Genomic DNA Translation: AAR93103.1
AY495137 Genomic DNA Translation: AAR93116.1
AY495138 Genomic DNA Translation: AAR93129.1
AY495139 Genomic DNA Translation: AAR93142.1
AY495140 Genomic DNA Translation: AAR93155.1
AY495141 Genomic DNA Translation: AAR93168.1
AY495142 Genomic DNA Translation: AAR93181.1
AY495143 Genomic DNA Translation: AAR93194.1
AY495144 Genomic DNA Translation: AAR93207.1
AY495145 Genomic DNA Translation: AAR93220.1
AY495146 Genomic DNA Translation: AAR93233.1
AY495147 Genomic DNA Translation: AAR93246.1
AY495148 Genomic DNA Translation: AAR93259.1
AY495149 Genomic DNA Translation: AAR93272.1
AY495150 Genomic DNA Translation: AAR93285.1
AY495151 Genomic DNA Translation: AAR93298.1
AY495152 Genomic DNA Translation: AAR93311.1
AY495153 Genomic DNA Translation: AAR93324.1
AY495154 Genomic DNA Translation: AAR93337.1
AY495155 Genomic DNA Translation: AAR93350.1
AY495156 Genomic DNA Translation: AAR93363.1
AY495157 Genomic DNA Translation: AAR93376.1
AY495158 Genomic DNA Translation: AAR93389.1
AY495159 Genomic DNA Translation: AAR93402.1
AY495160 Genomic DNA Translation: AAR93415.1
AY495161 Genomic DNA Translation: AAR93428.1
AY495162 Genomic DNA Translation: AAR93441.1
AY495163 Genomic DNA Translation: AAR93454.1
AY495164 Genomic DNA Translation: AAR93467.1
AY495165 Genomic DNA Translation: AAR93480.1
AY495167 Genomic DNA Translation: AAR93506.1
AY495168 Genomic DNA Translation: AAR93519.1
AY495169 Genomic DNA Translation: AAR93532.1
AY495171 Genomic DNA Translation: AAR93558.1
AY495172 Genomic DNA Translation: AAR93571.1
AY495173 Genomic DNA Translation: AAR93584.1
AY495174 Genomic DNA Translation: AAR93597.1
AY495175 Genomic DNA Translation: AAR93610.1
AY495176 Genomic DNA Translation: AAR93623.1
AY495177 Genomic DNA Translation: AAR93636.1
AY495178 Genomic DNA Translation: AAR93649.1
AY495179 Genomic DNA Translation: AAR93662.1
AY495180 Genomic DNA Translation: AAR93675.1
AY495181 Genomic DNA Translation: AAR93688.1
AY495182 Genomic DNA Translation: AAR93701.1
AY495183 Genomic DNA Translation: AAR93714.1
AY495184 Genomic DNA Translation: AAR93727.1
AY495185 Genomic DNA Translation: AAR93740.1
AY495186 Genomic DNA Translation: AAR93753.1
AY495187 Genomic DNA Translation: AAR93766.1
AY495188 Genomic DNA Translation: AAR93779.1
AY495189 Genomic DNA Translation: AAR93792.1
AY495190 Genomic DNA Translation: AAR93805.1
AY495191 Genomic DNA Translation: AAR93818.1
AY495192 Genomic DNA Translation: AAR93831.1
AY495193 Genomic DNA Translation: AAR93844.1
AY495194 Genomic DNA Translation: AAR93857.1
AY495239 Genomic DNA Translation: AAR94442.1
AY495240 Genomic DNA Translation: AAR94455.1
AY495241 Genomic DNA Translation: AAR94468.1
AY495242 Genomic DNA Translation: AAR94481.1
AY495244 Genomic DNA Translation: AAR94507.1
AY495246 Genomic DNA Translation: AAR94533.1
AY495247 Genomic DNA Translation: AAR94546.1
AY495248 Genomic DNA Translation: AAR94559.1
AY495249 Genomic DNA Translation: AAR94572.1
AY495252 Genomic DNA Translation: AAR94611.1
AY495267 Genomic DNA Translation: AAR94806.1
AY495268 Genomic DNA Translation: AAR94819.1
AY495269 Genomic DNA Translation: AAR94832.1
AY495270 Genomic DNA Translation: AAR94845.1
AY495271 Genomic DNA Translation: AAR94858.1
AY495272 Genomic DNA Translation: AAR94871.1
AY495273 Genomic DNA Translation: AAR94884.1
AY495274 Genomic DNA Translation: AAR94897.1
AY495275 Genomic DNA Translation: AAR94910.1
AY495276 Genomic DNA Translation: AAR94923.1
AY495277 Genomic DNA Translation: AAR94936.1
AY495278 Genomic DNA Translation: AAR94949.1
AY495279 Genomic DNA Translation: AAR94962.1
AY495280 Genomic DNA Translation: AAR94975.1
AY495281 Genomic DNA Translation: AAR94988.1
AY495282 Genomic DNA Translation: AAR95001.1
AY495283 Genomic DNA Translation: AAR95014.1
AY495284 Genomic DNA Translation: AAR95027.1
AY495285 Genomic DNA Translation: AAR95040.1
AY495286 Genomic DNA Translation: AAR95053.1
AY495287 Genomic DNA Translation: AAR95066.1
AY495288 Genomic DNA Translation: AAR95079.1
AY495289 Genomic DNA Translation: AAR95092.1
AY495290 Genomic DNA Translation: AAR95105.1
AY495291 Genomic DNA Translation: AAR95118.1
AY495292 Genomic DNA Translation: AAR95131.1
AY495293 Genomic DNA Translation: AAR95144.1
AY495294 Genomic DNA Translation: AAR95157.1
AY495295 Genomic DNA Translation: AAR95170.1
AY495297 Genomic DNA Translation: AAR95196.1
AY495298 Genomic DNA Translation: AAR95209.1
AY495299 Genomic DNA Translation: AAR95222.1
AY495300 Genomic DNA Translation: AAR95235.1
AY495301 Genomic DNA Translation: AAR95248.1
AY495302 Genomic DNA Translation: AAR95261.1
AY495303 Genomic DNA Translation: AAR95274.1
AY495304 Genomic DNA Translation: AAR95287.1
AY495305 Genomic DNA Translation: AAR95300.1
AY495306 Genomic DNA Translation: AAR95313.1
AY495307 Genomic DNA Translation: AAR95326.1
AY495308 Genomic DNA Translation: AAR95339.1
AY495309 Genomic DNA Translation: AAR95352.1
AY495310 Genomic DNA Translation: AAR95365.1
AY495311 Genomic DNA Translation: AAR95378.1
AY495312 Genomic DNA Translation: AAR95391.1
AY495313 Genomic DNA Translation: AAR95404.1
AY495314 Genomic DNA Translation: AAR95417.1
AY495315 Genomic DNA Translation: AAR95430.1
AY495316 Genomic DNA Translation: AAR95443.1
AY495317 Genomic DNA Translation: AAR95456.1
AY495318 Genomic DNA Translation: AAR95469.1
AY495319 Genomic DNA Translation: AAR95482.1
AY495320 Genomic DNA Translation: AAR95495.1
AY495321 Genomic DNA Translation: AAR95508.1
AY495322 Genomic DNA Translation: AAR95521.1
AY495323 Genomic DNA Translation: AAR95534.1
AY495324 Genomic DNA Translation: AAR95547.1
AY495325 Genomic DNA Translation: AAR95560.1
AY495326 Genomic DNA Translation: AAR95573.1
AY495327 Genomic DNA Translation: AAR95586.1
AY495328 Genomic DNA Translation: AAR95599.1
AY495329 Genomic DNA Translation: AAR95612.1
AY495330 Genomic DNA Translation: AAR95625.1
AF465942 Genomic DNA Translation: AAN14556.1
AF346963 Genomic DNA Translation: AAK17216.1
AF346964 Genomic DNA Translation: AAK17229.2
AF346966 Genomic DNA Translation: AAK17255.1
AF346967 Genomic DNA Translation: AAK17268.2
AF346968 Genomic DNA Translation: AAK17281.2
AF346969 Genomic DNA Translation: AAK17294.2
AF346971 Genomic DNA Translation: AAK17320.2
AF346974 Genomic DNA Translation: AAK17359.2
AF346975 Genomic DNA Translation: AAK17372.2
AF346977 Genomic DNA Translation: AAK17398.1
AF346978 Genomic DNA Translation: AAK17411.1
AF346980 Genomic DNA Translation: AAK17437.2
AF346981 Genomic DNA Translation: AAK17450.2
AF346982 Genomic DNA Translation: AAK17463.1
AF346985 Genomic DNA Translation: AAK17502.2
AF346986 Genomic DNA Translation: AAK17515.2
AF346987 Genomic DNA Translation: AAK17528.2
AF346989 Genomic DNA Translation: AAK17554.2
AF346990 Genomic DNA Translation: AAK17567.1
AF346993 Genomic DNA Translation: AAK17606.2
AF346994 Genomic DNA Translation: AAK17619.2
AF346996 Genomic DNA Translation: AAK17645.2
AF346997 Genomic DNA Translation: AAK17658.2
AF347000 Genomic DNA Translation: AAK17697.1
AF347002 Genomic DNA Translation: AAK17723.2
AF347003 Genomic DNA Translation: AAK17736.2
AF347004 Genomic DNA Translation: AAK17749.2
AF347005 Genomic DNA Translation: AAK17762.2
AF347006 Genomic DNA Translation: AAK17775.2
AF347007 Genomic DNA Translation: AAK17788.2
AF347010 Genomic DNA Translation: AAK17827.2
AF347011 Genomic DNA Translation: AAK17840.2
AF347012 Genomic DNA Translation: AAK17853.2
AF347013 Genomic DNA Translation: AAK17866.2
AF347014 Genomic DNA Translation: AAK17879.2
AF347015 Genomic DNA Translation: AAK17892.2
AY289051 Genomic DNA Translation: AAP47889.1
AY289052 Genomic DNA Translation: AAP47902.1
AY289055 Genomic DNA Translation: AAP47941.1
AY289056 Genomic DNA Translation: AAP47954.1
AY289058 Genomic DNA Translation: AAP47980.1
AY289059 Genomic DNA Translation: AAP47993.1
AY289060 Genomic DNA Translation: AAP48006.1
AY289061 Genomic DNA Translation: AAP48019.1
AY289062 Genomic DNA Translation: AAP48032.1
AY289063 Genomic DNA Translation: AAP48045.1
AY289065 Genomic DNA Translation: AAP48071.1
AY289068 Genomic DNA Translation: AAP48110.1
AY289069 Genomic DNA Translation: AAP48123.1
AY289070 Genomic DNA Translation: AAP48136.1
AY289071 Genomic DNA Translation: AAP48149.1
AY289073 Genomic DNA Translation: AAP48175.1
AY289074 Genomic DNA Translation: AAP48188.1
AY289075 Genomic DNA Translation: AAP48201.1
AY289076 Genomic DNA Translation: AAP48214.1
AY289077 Genomic DNA Translation: AAP48227.1
AY289079 Genomic DNA Translation: AAP48253.1
AY289080 Genomic DNA Translation: AAP48266.1
AY289081 Genomic DNA Translation: AAP48279.1
AY289082 Genomic DNA Translation: AAP48292.1
AY289083 Genomic DNA Translation: AAP48305.1
AY289084 Genomic DNA Translation: AAP48318.1
AY289085 Genomic DNA Translation: AAP48331.1
AY289087 Genomic DNA Translation: AAP48357.1
AY289088 Genomic DNA Translation: AAP48370.1
AY289090 Genomic DNA Translation: AAP48396.1
AY289091 Genomic DNA Translation: AAP48409.1
AY289092 Genomic DNA Translation: AAP48422.1
AY289093 Genomic DNA Translation: AAP48434.1
AY289094 Genomic DNA Translation: AAP48447.1
AY289095 Genomic DNA Translation: AAP48460.1
AY289096 Genomic DNA Translation: AAP48473.1
AY289097 Genomic DNA Translation: AAP48486.1
AY289098 Genomic DNA Translation: AAP48499.1
AY289099 Genomic DNA Translation: AAP48512.1
AY289100 Genomic DNA Translation: AAP48525.1
AY289101 Genomic DNA Translation: AAP48538.1
AY289102 Genomic DNA Translation: AAP48551.1
PIRiA00434 DNHUN4
RefSeqiYP_003024035.1, NC_012920.1

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTCelectron microscopy3.70r1-459[»]
5XTDelectron microscopy3.70r1-459[»]
ProteinModelPortaliP03905
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110634, 31 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP03905
IntActiP03905, 15 interactors
MINTiP03905
STRINGi9606.ENSP00000354961

Chemistry databases

BindingDBiP03905
ChEMBLiCHEMBL4499
DrugBankiDB00157 NADH

PTM databases

iPTMnetiP03905
PhosphoSitePlusiP03905

Polymorphism and mutation databases

DMDMi128748

Proteomic databases

EPDiP03905
PaxDbiP03905
PeptideAtlasiP03905
PRIDEiP03905
ProteomicsDBi51615

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000361381; ENSP00000354961; ENSG00000198886
GeneIDi4538
KEGGihsa:4538
UCSCiuc064xpj.1 human

Organism-specific databases

CTDi4538
DisGeNETi4538
EuPathDBiHostDB:ENSG00000198886.2
GeneCardsiMT-ND4
GeneReviewsiMT-ND4
H-InvDBiHIX0080294
HGNCiHGNC:7459 MT-ND4
HPAiHPA053928
MalaCardsiMT-ND4
MIMi500001 phenotype
516003 gene
535000 phenotype
540000 phenotype
neXtProtiNX_P03905
OpenTargetsiENSG00000198886
Orphaneti104 Leber hereditary optic neuropathy
99718 Leber plus disease
255210 Maternally-inherited Leigh syndrome
550 MELAS
168609 Mitochondrial non-syndromic sensorineural deafness with susceptibility to aminoglycoside exposure
PharmGKBiPA31263
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4845 Eukaryota
COG1008 LUCA
GeneTreeiENSGT00730000111316
HOGENOMiHOG000100683
HOVERGENiHBG093667
InParanoidiP03905
KOiK03881
OMAiITRWGNQ
OrthoDBiEOG091G0T7G
PhylomeDBiP03905
TreeFamiTF343520

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis

Miscellaneous databases

ChiTaRSiND4 human
GeneWikiiMT-ND4
GenomeRNAii4538
PROiPR:P03905
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000198886 Expressed in 203 organ(s), highest expression level in right adrenal gland
ExpressionAtlasiP03905 baseline and differential
GenevisibleiP03905 HS

Family and domain databases

InterProiView protein in InterPro
IPR000260 NADH4_N
IPR010227 NADH_Q_OxRdtase_chainM/4
IPR003918 NADH_UbQ_OxRdtase
IPR001750 ND/Mrp_mem
PANTHERiPTHR43507 PTHR43507, 1 hit
PfamiView protein in Pfam
PF01059 Oxidored_q5_N, 1 hit
PF00361 Proton_antipo_M, 1 hit
PRINTSiPR01437 NUOXDRDTASE4
TIGRFAMsiTIGR01972 NDH_I_M, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiNU4M_HUMAN
AccessioniPrimary (citable) accession number: P03905
Secondary accession number(s): Q6RL39, Q6RQN9, Q8HNR8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 21, 1986
Last modified: November 7, 2018
This is version 181 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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