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Protein

NADH-ubiquinone oxidoreductase chain 4

Gene

MT-ND4

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Keywords - Ligandi

NAD, Ubiquinone

Enzyme and pathway databases

BioCyciZFISH:HS00033-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Names & Taxonomyi

Protein namesi
Recommended name:
NADH-ubiquinone oxidoreductase chain 4 (EC:1.6.5.3)
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

HGNCiHGNC:7459. MT-ND4.

Subcellular locationi

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

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Leber hereditary optic neuropathy (LHON)3 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 rs199476112dbSNPEnsembl.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 rs200873900dbSNPEnsembl.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 rs199476113dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation, Dystonia, Leber hereditary optic neuropathy, MELAS syndrome

Organism-specific databases

DisGeNETi4538.
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.

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.

PTM databases

iPTMnetiP03905.
PhosphoSitePlusiP03905.

Expressioni

Gene expression databases

BgeeiENSG00000198886.
ExpressionAtlasiP03905. baseline and differential.
GenevisibleiP03905. HS.

Organism-specific databases

HPAiHPA053928.

Interactioni

Protein-protein interaction databases

BioGridi110634. 24 interactors.
IntActiP03905. 14 interactors.
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.
OMAiIQTPWSY.
OrthoDBiEOG091G0T7G.
PhylomeDBiP03905.
TreeFamiTF343520.

Family and domain databases

InterProiIPR000260. NADH4_N.
IPR010227. NADH_Q_OxRdtase_chainM/4.
IPR003918. NADH_UbQ_OxRdtase.
IPR001750. ND/Mrp_mem.
[Graphical view]
PfamiPF01059. Oxidored_q5_N. 1 hit.
PF00361. Proton_antipo_M. 1 hit.
[Graphical view]
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 → P.2 Publications1
Natural variantiVAR_004759109T → A in MELAS. 1 PublicationCorresponds to variant rs199476113dbSNPEnsembl.1
Natural variantiVAR_008600109T → P.1 Publication1
Natural variantiVAR_008601132I → T.1 Publication1
Natural variantiVAR_008602294M → T.1 PublicationCorresponds to variant rs193302940dbSNPEnsembl.1
Natural variantiVAR_008393313V → I in LDYT; possible rare primary mutation. 1 PublicationCorresponds to variant rs200873900dbSNPEnsembl.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 rs199476112dbSNPEnsembl.1
Natural variantiVAR_064565420T → A Detected in a patient with mitochondrial complex I deficiency; uncertain pathological significance. 1 Publication1

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

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

ProteinModelPortaliP03905.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110634. 24 interactors.
IntActiP03905. 14 interactors.
STRINGi9606.ENSP00000354961.

Chemistry databases

BindingDBiP03905.
ChEMBLiCHEMBL4499.

PTM databases

iPTMnetiP03905.
PhosphoSitePlusiP03905.

Polymorphism and mutation databases

DMDMi128748.

Proteomic databases

EPDiP03905.
PaxDbiP03905.
PeptideAtlasiP03905.
PRIDEiP03905.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi4538.
DisGeNETi4538.
GeneCardsiMT-ND4.
GeneReviewsiMT-ND4.
H-InvDBHIX0080294.
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.
OMAiIQTPWSY.
OrthoDBiEOG091G0T7G.
PhylomeDBiP03905.
TreeFamiTF343520.

Enzyme and pathway databases

BioCyciZFISH:HS00033-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Miscellaneous databases

GeneWikiiMT-ND4.
GenomeRNAii4538.
PROiP03905.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000198886.
ExpressionAtlasiP03905. baseline and differential.
GenevisibleiP03905. HS.

Family and domain databases

InterProiIPR000260. NADH4_N.
IPR010227. NADH_Q_OxRdtase_chainM/4.
IPR003918. NADH_UbQ_OxRdtase.
IPR001750. ND/Mrp_mem.
[Graphical view]
PfamiPF01059. Oxidored_q5_N. 1 hit.
PF00361. Proton_antipo_M. 1 hit.
[Graphical view]
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 historyi
Integrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 21, 1986
Last modified: November 2, 2016
This is version 168 of the entry and version 1 of the sequence. [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

Complete proteome, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.