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P03905 (NU4M_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified March 19, 2014. Version 140. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (4) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
NADH-ubiquinone oxidoreductase chain 4

EC=1.6.5.3
Alternative name(s):
NADH dehydrogenase subunit 4
Gene names
Name:MT-ND4
Synonyms:MTND4, NADH4, ND4
Encoded onMitochondrion
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length459 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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.

Catalytic activity

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

Subcellular location

Mitochondrion membrane; Multi-pass membrane protein By similarity.

Involvement in disease

Leber hereditary optic neuropathy (LHON) [MIM:535000]: A 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.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.12 Ref.13 Ref.14 Ref.18

Leber hereditary optic neuropathy with dystonia (LDYT) [MIM:500001]: Part of a spectrum of Leber hereditary optic neuropathy. It is characterized by the association of optic atrophy and central vision loss with dystonia.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.17

Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]: Genetically heterogeneous disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.16

Sequence similarities

Belongs to the complex I subunit 4 family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 459459NADH-ubiquinone oxidoreductase chain 4
PRO_0000117942

Regions

Transmembrane22 – 4221Helical; Potential
Transmembrane60 – 8021Helical; Potential
Transmembrane102 – 12221Helical; Potential
Transmembrane145 – 16521Helical; Potential
Transmembrane196 – 21621Helical; Potential
Transmembrane224 – 24421Helical; Potential
Transmembrane257 – 27721Helical; Potential
Transmembrane284 – 30320Helical; Potential
Transmembrane308 – 33023Helical; Potential
Transmembrane351 – 37121Helical; Potential
Transmembrane391 – 41121Helical; Potential

Natural variations

Natural variant791A → P. Ref.3 Ref.15
VAR_008599
Natural variant1091T → A in MELAS. Ref.16
VAR_004759
Natural variant1091T → P. Ref.15
VAR_008600
Natural variant1321I → T. Ref.15
VAR_008601
Natural variant2941M → T. Ref.15
VAR_008602
Natural variant3131V → I in LDYT; possible rare primary mutation. Ref.17
VAR_008393
Natural variant3401R → 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. Ref.12 Ref.13 Ref.14 Ref.18
VAR_004760
Natural variant4201T → A Detected in a patient with mitochondrial complex I deficiency; uncertain pathological significance. Ref.19
VAR_064565

Experimental info

Sequence conflict2301V → L Ref.2

Sequences

Sequence LengthMass (Da)Tools
P03905 [UniParc].

Last modified July 21, 1986. Version 1.
Checksum: 2EEE581F3BD270AF

FASTA45951,581
        10         20         30         40         50         60 
MLKLIVPTIM LLPLTWLSKK HMIWINTTTH SLIISIIPLL FFNQINNNLF SCSPTFSSDP 

        70         80         90        100        110        120 
LTTPLLMLTT WLLPLTIMAS QRHLSSEPLS RKKLYLSMLI SLQISLIMTF TATELIMFYI 

       130        140        150        160        170        180 
FFETTLIPTL AIITRWGNQP ERLNAGTYFL FYTLVGSLPL LIALIYTHNT LGSLNILLLT 

       190        200        210        220        230        240 
LTAQELSNSW ANNLMWLAYT MAFMVKMPLY GLHLWLPKAH VEAPIAGSMV LAAVLLKLGG 

       250        260        270        280        290        300 
YGMMRLTLIL NPLTKHMAYP FLVLSLWGMI MTSSICLRQT DLKSLIAYSS ISHMALVVTA 

       310        320        330        340        350        360 
ILIQTPWSFT GAVILMIAHG LTSSLLFCLA NSNYERTHSR IMILSQGLQT LLPLMAFWWL 

       370        380        390        400        410        420 
LASLANLALP PTINLLGELS VLVTTFSWSN ITLLLTGLNM LVTALYSLYM FTTTQWGSLT 

       430        440        450 
HHINNMKPSF TRENTLMFMH LSPILLLSLN PDIITGFSS 

« Hide

References

« Hide 'large scale' references
[1]"Sequence and organization of the human mitochondrial genome."
Anderson S., Bankier A.T., Barrell B.G., de Bruijn M.H.L., Coulson A.R., Drouin J., Eperon I.C., Nierlich D.P., Roe B.A., Sanger F., Schreier P.H., Smith A.J.H., Staden R., Young I.G.
Nature 290:457-465(1981) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[2]"Differentiation of HT-29 human colonic adenocarcinoma cells correlates with increased expression of mitochondrial RNA: effects of trehalose on cell growth and maturation."
Lu X., Walker T., Macmanus J.P., Seligy V.L.
Cancer Res. 52:3718-3725(1992) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]"Mitochondrial genome diversity of native Americans supports a single early entry of founder populations into America."
Silva W.A. Jr., Bonatto S.L., Holanda A.J., Ribeiro-Dos-Santos A.K., Paixao B.M., Goldman G.H., Abe-Sandes K., Rodriguez-Delfin L., Barbosa M., Paco-Larson M.L., Petzl-Erler M.L., Valente V., Santos S.E., Zago M.A.
Am. J. Hum. Genet. 71:187-192(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT PRO-79.
[4]"Lineage-specific selection in human mtDNA: lack of polymorphisms in a segment of MTND5 gene in haplogroup J."
Moilanen J.S., Finnila S., Majamaa K.
Mol. Biol. Evol. 20:2132-2142(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[5]"Mitochondrial genome variation and the origin of modern humans."
Ingman M., Kaessmann H., Paeaebo S., Gyllensten U.
Nature 408:708-713(2000) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[6]"Mitochondrial genome variation and evolutionary history of Australian and New Guinean aborigines."
Ingman M., Gyllensten U.
Genome Res. 13:1600-1606(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[7]"Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians."
Coble M.D., Just R.S., O'Callaghan J.E., Letmanyi I.H., Peterson C.T., Irwin J.A., Parsons T.J.
Int. J. Legal Med. 118:137-146(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[8]"Mitochondrial DNA sequences of primates: tempo and mode of evolution."
Brown W.M., Prager E.M., Wang A., Wilson A.C.
J. Mol. Evol. 18:225-239(1982) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 308-459.
[9]"Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase."
Chomyn A., Mariottini P., Cleeter M.W.J., Ragan C.I., Matsuno-Yagi A., Hatefi Y., Doolittle R.F., Attardi G.
Nature 314:592-597(1985) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION OF PROTEIN.
[10]"The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification."
Murray J., Zhang B., Taylor S.W., Oglesbee D., Fahy E., Marusich M.F., Ghosh S.S., Capaldi R.A.
J. Biol. Chem. 278:13619-13622(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: MASS SPECTROMETRY, IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX.
[11]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[12]"Mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy."
Wallace D.C., Singh G., Lott M.T., Hodge J.A., Schurr T.G., Lezza A.M., Elsas L.J. II, Nikoskelainen E.K.
Science 242:1427-1430(1988) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON HIS-340.
[13]"Electron transfer properties of NADH:ubiquinone reductase in the ND1/3460 and the ND4/11778 mutations of the Leber hereditary optic neuroretinopathy (LHON)."
Majander A., Huoponen K., Savontaus M.-L., Nikoskelainen E., Wikstroem M.
FEBS Lett. 292:289-292(1991) [PubMed] [Europe PMC] [Abstract]
Cited for: CHARACTERIZATION OF VARIANT LHON HIS-340.
[14]"Detection of the G to A mitochondrial DNA mutation at position 11778 in German families with Leber's hereditary optic neuropathy."
Kormann B.A., Schuster H., Berninger T.A., Leo-Kottler B.
Hum. Genet. 88:98-100(1991) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON HIS-340.
[15]"Normal variants of human mitochondrial DNA and translation products: the building of a reference data base."
Marzuki S., Noer A.S., Lertrit P., Thyagarajan D., Kapsa R., Utthanaphol P., Byrne E.
Hum. Genet. 88:139-145(1991) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANTS PRO-79; PRO-109; THR-132 AND THR-294.
[16]"A new disease-related mutation for mitochondrial encephalopathy lactic acidosis and strokelike episodes (MELAS) syndrome affects the ND4 subunit of the respiratory complex I."
Lertrit P., Noer A.S., Jean-Francois M.J.B., Kapsa R., Dennett X., Thyagarajan D., Lethlean K., Byrne E., Marzuki S.
Am. J. Hum. Genet. 51:457-468(1992) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT MELAS ALA-109.
[17]"Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia."
de Vries D.D., Went L.N., Bruyn G.W., Scholte H.R., Hofstra R.M.W., Bolhuis P.A., van Oost B.A.
Am. J. Hum. Genet. 58:703-711(1996) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LDYT ILE-313.
[18]"Leber's hereditary optic neuropathy in Indonesia: two families with the mtDNA 11778G>A and 14484T>C mutations."
Sudoyo H., Sitepu M., Malik S., Poesponegoro H.D., Marzuki S.
Hum. Mutat. Suppl. 1:S271-S274(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON HIS-340.
[19]"High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency."
Calvo S.E., Tucker E.J., Compton A.G., Kirby D.M., Crawford G., Burtt N.P., Rivas M., Guiducci C., Bruno D.L., Goldberger O.A., Redman M.C., Wiltshire E., Wilson C.J., Altshuler D., Gabriel S.B., Daly M.J., Thorburn D.R., Mootha V.K.
Nat. Genet. 42:851-858(2010) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT ALA-420.
+Additional computationally mapped references.

Web resources

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
PIRDNHUN4. A00434.
RefSeqYP_003024035.1. NC_012920.1.

3D structure databases

ProteinModelPortalP03905.
SMRP03905. Positions 2-458.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid110634. 4 interactions.
IntActP03905. 4 interactions.

Chemistry

BindingDBP03905.
ChEMBLCHEMBL4499.
DrugBankDB00157. NADH.

PTM databases

PhosphoSiteP03905.

Polymorphism databases

DMDM128748.

Proteomic databases

PaxDbP03905.
PeptideAtlasP03905.
PRIDEP03905.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000361381; ENSP00000354961; ENSG00000198886.
GeneID4538.
KEGGhsa:4538.

Organism-specific databases

CTD4538.
GeneCardsGCMTP010762.
H-InvDBHIX0080294.
HGNCHGNC:7459. MT-ND4.
HPAHPA053928.
MIM500001. phenotype.
516003. gene.
535000. phenotype.
540000. phenotype.
neXtProtNX_P03905.
Orphanet99718. Leber 'plus' disease.
104. Leber hereditary optic neuropathy.
255210. Maternally-inherited Leigh syndrome.
550. MELAS syndrome.
168609. Mitochondrial nonsyndromic sensorineural deafness with susceptibility to aminoglycoside exposure.
PharmGKBPA31263.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG1008.
HOGENOMHOG000100683.
HOVERGENHBG093667.
InParanoidP03905.
KOK03881.
OMAPNMIWIN.
OrthoDBEOG7DJSMN.
PhylomeDBP03905.
ProtClustDBMTH00110.
TreeFamTF343520.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

ArrayExpressP03905.
BgeeP03905.
GenevestigatorP03905.

Family and domain databases

InterProIPR010227. NADH_Q_OxRdtase_chainM/4.
IPR001750. NADH_UbQ/plastoQ_OxRdtase.
IPR003918. NADH_UbQ_OxRdtase.
IPR000260. NADH_UbQ_OxRdtase_chain4_N.
[Graphical view]
PfamPF00361. Oxidored_q1. 1 hit.
PF01059. Oxidored_q5_N. 1 hit.
[Graphical view]
PRINTSPR01437. NUOXDRDTASE4.
TIGRFAMsTIGR01972. NDH_I_M. 1 hit.
ProtoNetSearch...

Other

GeneWikiMT-ND4.
GenomeRNAi4538.
NextBio17505.
PROP03905.
SOURCESearch...

Entry information

Entry nameNU4M_HUMAN
AccessionPrimary (citable) accession number: P03905
Secondary accession number(s): Q6RL39, Q6RQN9, Q8HNR8
Entry history
Integrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 21, 1986
Last modified: March 19, 2014
This is version 140 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations