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P03915 (NU5M_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 145. 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 5

EC=1.6.5.3
Alternative name(s):
NADH dehydrogenase subunit 5
Gene names
Name:MT-ND5
Synonyms:MTND5, NADH5, ND5
Encoded onMitochondrion
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length603 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 inner 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.13 Ref.14 Ref.15 Ref.21

Leigh syndrome (LS) [MIM:256000]: An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.18 Ref.20 Ref.23

Mitochondrial complex I deficiency (MT-C1D) [MIM:252010]: A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.24

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 Ref.19 Ref.22 Ref.23

Sequence similarities

Belongs to the complex I subunit 5 family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 603603NADH-ubiquinone oxidoreductase chain 5
PRO_0000118101

Regions

Transmembrane38 – 5821Helical; Potential
Transmembrane87 – 10721Helical; Potential
Transmembrane122 – 14221Helical; Potential
Transmembrane144 – 16017Helical; Potential
Transmembrane171 – 19121Helical; Potential
Transmembrane211 – 23323Helical; Potential
Transmembrane241 – 26121Helical; Potential
Transmembrane272 – 29221Helical; Potential
Transmembrane301 – 32020Helical; Potential
Transmembrane325 – 34723Helical; Potential
Transmembrane370 – 39021Helical; Potential
Transmembrane407 – 42923Helical; Potential
Transmembrane458 – 47821Helical; Potential
Transmembrane482 – 50221Helical; Potential
Transmembrane582 – 60221Helical; Potential

Natural variations

Natural variant171P → S. Ref.12
VAR_008864
Natural variant951F → S. Ref.12
VAR_008865
Natural variant991S → P. Ref.12
VAR_008866
Natural variant1241F → L in LS and MT-C1D. Ref.18 Ref.24
VAR_035424
Natural variant1451E → G in MELAS. Ref.19
VAR_035425
Natural variant1461G → D. Ref.12
VAR_008867
Natural variant1601A → V. Ref.12
VAR_008868
Natural variant1651N → S. Ref.12
VAR_008869
Natural variant1711A → V in LHON. Ref.21
VAR_035426
Natural variant2111T → P. Ref.3
VAR_011357
Natural variant2361A → T in MELAS/LS; due to mitochondrial complex I deficiency. Ref.22 Ref.23
VAR_035427
Natural variant2371M → L in MELAS/LHON/LS; due to mitochondrial complex I deficiency. Ref.19
VAR_035428
Natural variant2501S → C in MELAS/LS; due to mitochondrial complex I deficiency. Ref.20
VAR_035429
Natural variant2531V → A in MT-C1D. Ref.24
VAR_064566
Natural variant2571I → V. Ref.3 Ref.4
Corresponds to variant rs2853501 [ dbSNP | Ensembl ].
VAR_011358
Natural variant3041F → S. Ref.12
VAR_008870
Natural variant3141M → V. Ref.3
Corresponds to variant rs2853502 [ dbSNP | Ensembl ].
VAR_011359
Natural variant3311T → A. Ref.12
VAR_008871
Natural variant3931D → N in MELAS and MT-C1D. Ref.16 Ref.23 Ref.24
VAR_035430
Natural variant4471N → S Detected in a patient with mitochondrial complex I deficiency; uncertain pathological significance. Ref.24
VAR_064567
Natural variant4581A → T in LHON; secondary mutation; does not seem to directly cause the disease. Ref.13 Ref.14
Corresponds to variant rs28359178 [ dbSNP | Ensembl ].
VAR_004761
Natural variant4651G → E in LHON; primary rare mutation. Ref.15
VAR_004762
Natural variant4751A → T. Ref.17
VAR_008603
Natural variant5031D → G. Ref.12
VAR_008872

Experimental info

Sequence conflict4561R → G in CAA24036. Ref.1

Sequences

Sequence LengthMass (Da)Tools
P03915 [UniParc].

Last modified May 30, 2000. Version 2.
Checksum: 18A06227ABC9162A

FASTA60367,027
        10         20         30         40         50         60 
MTMHTTMTTL TLTSLIPPIL TTLVNPNKKN SYPHYVKSIV ASTFIISLFP TTMFMCLDQE 

        70         80         90        100        110        120 
VIISNWHWAT TQTTQLSLSF KLDYFSMMFI PVALFVTWSI MEFSLWYMNS DPNINQFFKY 

       130        140        150        160        170        180 
LLIFLITMLI LVTANNLFQL FIGWEGVGIM SFLLISWWYA RADANTAAIQ AILYNRIGDI 

       190        200        210        220        230        240 
GFILALAWFI LHSNSWDPQQ MALLNANPSL TPLLGLLLAA AGKSAQLGLH PWLPSAMEGP 

       250        260        270        280        290        300 
TPVSALLHSS TMVVAGIFLL IRFHPLAENS PLIQTLTLCL GAITTLFAAV CALTQNDIKK 

       310        320        330        340        350        360 
IVAFSTSSQL GLMMVTIGIN QPHLAFLHIC THAFFKAMLF MCSGSIIHNL NNEQDIRKMG 

       370        380        390        400        410        420 
GLLKTMPLTS TSLTIGSLAL AGMPFLTGFY SKDHIIETAN MSYTNAWALS ITLIATSLTS 

       430        440        450        460        470        480 
AYSTRMILLT LTGQPRFPTL TNINENNPTL LNPIKRLAAG SLFAGFLITN NISPASPFQT 

       490        500        510        520        530        540 
TIPLYLKLTA LAVTFLGLLT ALDLNYLTNK LKMKSPLCTF YFSNMLGFYP SITHRTIPYL 

       550        560        570        580        590        600 
GLLTSQNLPL LLLDLTWLEK LLPKTISQHQ ISTSIITSTQ KGMIKLYFLS FFFPLILTLL 


LIT 

« 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]Kogelnik A., Brown M.
Submitted (APR-1997) to the EMBL/GenBank/DDBJ databases
Cited for: SEQUENCE REVISION TO 456.
[3]"Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs."
Horai S., Hayasaka K., Kondo R., Tsugane K., Takahata N.
Proc. Natl. Acad. Sci. U.S.A. 92:532-536(1995) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS PRO-211; VAL-257 AND VAL-314.
Tissue: Placenta.
[4]"Major genomic mitochondrial lineages delineate early human expansions."
Maca-Meyer N., Gonzalez A.M., Larruga J.M., Flores C., Cabrera V.M.
BMC Genet. 2:13-13(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT VAL-257.
[5]"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].
[6]"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].
[7]"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].
[8]"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].
[9]"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 1-79.
[10]"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.
[11]"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: IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX, IDENTIFICATION BY MASS SPECTROMETRY.
[12]"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 SER-17; SER-95; PRO-99; ASP-146; VAL-160; SER-165; SER-304; ALA-331 AND GLY-503.
[13]"Mitochondrial DNA complex I and III mutations associated with Leber's hereditary optic neuropathy."
Brown M.D., Voljavec A.S., Lott M.T., Torroni A., Yang C.C., Wallace D.C.
Genetics 130:163-173(1992) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON THR-458.
[14]"Alternative, simultaneous complex I mitochondrial DNA mutations in Leber's hereditary optic neuropathy."
Johns D.R., Berman J.
Biochem. Biophys. Res. Commun. 174:1324-1330(1991) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON THR-458.
[15]"When does bilateral optic atrophy become Leber hereditary optic neuropathy?"
Howell N., Halvorson S., Burns J., McCullough D.A., Poulton J.
Am. J. Hum. Genet. 53:959-963(1993) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON GLU-465.
[16]"Identification of a novel mutation in the mtDNA ND5 gene associated with MELAS."
Santorelli F.M., Tanji K., Kulikova R., Shanske S., Vilarinho L., Hays A.P., DiMauro S.
Biochem. Biophys. Res. Commun. 238:326-328(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT MELAS ASN-393.
[17]"Automating the identification of DNA variations using quality-based fluorescence re-sequencing: analysis of the human mitochondrial genome."
Rieder M.J., Taylor S.L., Tobe V.O., Nickerson D.A.
Nucleic Acids Res. 26:967-973(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT THR-475.
[18]"Leigh disease associated with a novel mitochondrial DNA ND5 mutation."
Taylor R.W., Morris A.A.M., Hutchinson M., Turnbull D.M.
Eur. J. Hum. Genet. 10:141-144(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LS LEU-124.
[19]"Is the mitochondrial complex I ND5 gene a hot-spot for MELAS causing mutations?"
Liolitsa D., Rahman S., Benton S., Carr L.J., Hanna M.G.
Ann. Neurol. 53:128-132(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANTS MELAS GLY-145 AND LEU-237.
[20]"A missense mutation in the mitochondrial ND5 gene associated with a Leigh-MELAS overlap syndrome."
Crimi M., Galbiati S., Moroni I., Bordoni A., Perini M.P., Lamantea E., Sciacco M., Zeviani M., Biunno I., Moggio M., Scarlato G., Comi G.P.
Neurology 60:1857-1861(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LS CYS-250.
[21]"The role of the ND5 gene in LHON: characterization of a new, heteroplasmic LHON mutation."
Mayorov V., Biousse V., Newman N.J., Brown M.D.
Ann. Neurol. 58:807-811(2005) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON VAL-171.
[22]"Novel mitochondrial DNA ND5 mutation in a patient with clinical features of MELAS and MERRF."
Naini A.B., Lu J., Kaufmann P., Bernstein R.A., Mancuso M., Bonilla E., Hirano M., DiMauro S.
Arch. Neurol. 62:473-476(2005) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT MELAS THR-236.
[23]"Mutations in the ND5 subunit of complex I of the mitochondrial DNA are a frequent cause of oxidative phosphorylation disease."
Blok M.J., Spruijt L., de Coo I.F.M., Schoonderwoerd K., Hendrickx A., Smeets H.J.
J. Med. Genet. 44:E74-E74(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LS THR-236, VARIANT MELAS ASN-393.
[24]"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: VARIANTS MT-C1D LEU-124; ALA-253 AND ASN-393, VARIANT SER-447.

Web resources

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
J01415 Genomic DNA. Translation: AAB58953.1.
V00662 Genomic DNA. Translation: CAA24036.1.
D38112 Genomic DNA. Translation: BAA07297.1.
AF465942 Genomic DNA. Translation: AAN14557.1.
AY339402 Genomic DNA. Translation: AAP89046.1.
AY339403 Genomic DNA. Translation: AAP89059.1.
AY339404 Genomic DNA. Translation: AAP89072.1.
AY339405 Genomic DNA. Translation: AAP89085.1.
AY339406 Genomic DNA. Translation: AAP89098.1.
AY339407 Genomic DNA. Translation: AAP89111.1.
AY339408 Genomic DNA. Translation: AAP89124.1.
AY339409 Genomic DNA. Translation: AAP89137.1.
AY339410 Genomic DNA. Translation: AAP89150.1.
AY339411 Genomic DNA. Translation: AAP89163.1.
AY339412 Genomic DNA. Translation: AAP89176.1.
AY339413 Genomic DNA. Translation: AAP89189.1.
AY339414 Genomic DNA. Translation: AAP89202.1.
AY339415 Genomic DNA. Translation: AAP89215.1.
AY339416 Genomic DNA. Translation: AAP89228.1.
AY339417 Genomic DNA. Translation: AAP89241.1.
AY339418 Genomic DNA. Translation: AAP89254.1.
AY339419 Genomic DNA. Translation: AAP89267.1.
AY339420 Genomic DNA. Translation: AAP89280.1.
AY339421 Genomic DNA. Translation: AAP89293.1.
AY339422 Genomic DNA. Translation: AAP89306.1.
AY339423 Genomic DNA. Translation: AAP89319.1.
AY339424 Genomic DNA. Translation: AAP89332.1.
AY339425 Genomic DNA. Translation: AAP89345.1.
AY339426 Genomic DNA. Translation: AAP89358.1.
AY339427 Genomic DNA. Translation: AAP89371.1.
AY339428 Genomic DNA. Translation: AAP89384.1.
AY339429 Genomic DNA. Translation: AAP89397.1.
AY339430 Genomic DNA. Translation: AAP89410.1.
AY339431 Genomic DNA. Translation: AAP89423.1.
AY339432 Genomic DNA. Translation: AAP89436.1.
AY339433 Genomic DNA. Translation: AAP89449.1.
AY339434 Genomic DNA. Translation: AAP89462.1.
AY339435 Genomic DNA. Translation: AAP89475.1.
AY339436 Genomic DNA. Translation: AAP89488.1.
AY339437 Genomic DNA. Translation: AAP89501.1.
AY339438 Genomic DNA. Translation: AAP89514.1.
AY339439 Genomic DNA. Translation: AAP89527.1.
AY339440 Genomic DNA. Translation: AAP89540.1.
AY339441 Genomic DNA. Translation: AAP89553.1.
AY339442 Genomic DNA. Translation: AAP89566.1.
AY339443 Genomic DNA. Translation: AAP89579.1.
AY339444 Genomic DNA. Translation: AAP89592.1.
AY339445 Genomic DNA. Translation: AAP89605.1.
AY339446 Genomic DNA. Translation: AAP89618.1.
AY339447 Genomic DNA. Translation: AAP89631.1.
AY339448 Genomic DNA. Translation: AAP89644.1.
AY339449 Genomic DNA. Translation: AAP89657.1.
AY339451 Genomic DNA. Translation: AAP89683.1.
AY339452 Genomic DNA. Translation: AAP89696.1.
AY339453 Genomic DNA. Translation: AAP89709.1.
AY339454 Genomic DNA. Translation: AAP89722.1.
AY339455 Genomic DNA. Translation: AAP89735.1.
AY339456 Genomic DNA. Translation: AAP89748.1.
AY339457 Genomic DNA. Translation: AAP89761.1.
AY339458 Genomic DNA. Translation: AAP89774.1.
AY339459 Genomic DNA. Translation: AAP89787.1.
AY339460 Genomic DNA. Translation: AAP89800.1.
AY339461 Genomic DNA. Translation: AAP89813.1.
AY339462 Genomic DNA. Translation: AAP89826.1.
AY339463 Genomic DNA. Translation: AAP89839.1.
AY339464 Genomic DNA. Translation: AAP89852.1.
AY339465 Genomic DNA. Translation: AAP89865.1.
AY339466 Genomic DNA. Translation: AAP89878.1.
AY339467 Genomic DNA. Translation: AAP89891.1.
AY339468 Genomic DNA. Translation: AAP89904.1.
AY339469 Genomic DNA. Translation: AAP89917.1.
AY339470 Genomic DNA. Translation: AAP89930.1.
AY339471 Genomic DNA. Translation: AAP89943.1.
AY339472 Genomic DNA. Translation: AAP89956.1.
AY339473 Genomic DNA. Translation: AAP89969.1.
AY339474 Genomic DNA. Translation: AAP89982.1.
AY339475 Genomic DNA. Translation: AAP89995.1.
AY339476 Genomic DNA. Translation: AAP90008.1.
AY339477 Genomic DNA. Translation: AAP90021.1.
AY339478 Genomic DNA. Translation: AAP90034.1.
AY339479 Genomic DNA. Translation: AAP90047.1.
AY339480 Genomic DNA. Translation: AAP90060.1.
AY339481 Genomic DNA. Translation: AAP90073.1.
AY339482 Genomic DNA. Translation: AAP90086.1.
AY339483 Genomic DNA. Translation: AAP90099.1.
AY339484 Genomic DNA. Translation: AAP90112.1.
AY339485 Genomic DNA. Translation: AAP90125.1.
AY339486 Genomic DNA. Translation: AAP90138.1.
AY339487 Genomic DNA. Translation: AAP90151.1.
AY339488 Genomic DNA. Translation: AAP90164.1.
AY339489 Genomic DNA. Translation: AAP90177.1.
AY339490 Genomic DNA. Translation: AAP90190.1.
AY339491 Genomic DNA. Translation: AAP90203.1.
AY339492 Genomic DNA. Translation: AAP90216.1.
AY339493 Genomic DNA. Translation: AAP90229.1.
AY339494 Genomic DNA. Translation: AAP90242.1.
AY339495 Genomic DNA. Translation: AAP90255.1.
AY339496 Genomic DNA. Translation: AAP90268.1.
AY339514 Genomic DNA. Translation: AAP90502.1.
AY339515 Genomic DNA. Translation: AAP90515.1.
AY339516 Genomic DNA. Translation: AAP90528.1.
AY339517 Genomic DNA. Translation: AAP90541.1.
AY339518 Genomic DNA. Translation: AAP90554.1.
AY339519 Genomic DNA. Translation: AAP90567.1.
AY339520 Genomic DNA. Translation: AAP90580.1.
AY339521 Genomic DNA. Translation: AAP90593.1.
AY339522 Genomic DNA. Translation: AAP90606.1.
AY339523 Genomic DNA. Translation: AAP90619.1.
AY339528 Genomic DNA. Translation: AAP90684.1.
AY339529 Genomic DNA. Translation: AAP90697.1.
AY339530 Genomic DNA. Translation: AAP90710.1.
AY339531 Genomic DNA. Translation: AAP90723.1.
AY339532 Genomic DNA. Translation: AAP90736.1.
AY339533 Genomic DNA. Translation: AAP90749.1.
AY339534 Genomic DNA. Translation: AAP90762.1.
AY339535 Genomic DNA. Translation: AAP90775.1.
AY339536 Genomic DNA. Translation: AAP90788.1.
AY339537 Genomic DNA. Translation: AAP90801.1.
AY339538 Genomic DNA. Translation: AAP90814.1.
AY339539 Genomic DNA. Translation: AAP90827.1.
AY339540 Genomic DNA. Translation: AAP90840.1.
AY339541 Genomic DNA. Translation: AAP90853.1.
AY339542 Genomic DNA. Translation: AAP90866.1.
AY339543 Genomic DNA. Translation: AAP90879.1.
AY339547 Genomic DNA. Translation: AAP90931.1.
AY339548 Genomic DNA. Translation: AAP90944.1.
AY339549 Genomic DNA. Translation: AAP90957.1.
AY339550 Genomic DNA. Translation: AAP90970.1.
AY339554 Genomic DNA. Translation: AAP91022.1.
AY339555 Genomic DNA. Translation: AAP91035.1.
AY339556 Genomic DNA. Translation: AAP91048.1.
AY339557 Genomic DNA. Translation: AAP91061.1.
AY339558 Genomic DNA. Translation: AAP91074.1.
AY339559 Genomic DNA. Translation: AAP91087.1.
AY339560 Genomic DNA. Translation: AAP91100.1.
AY339563 Genomic DNA. Translation: AAP91139.1.
AY339564 Genomic DNA. Translation: AAP91152.1.
AY339565 Genomic DNA. Translation: AAP91165.1.
AY339566 Genomic DNA. Translation: AAP91178.1.
AY339567 Genomic DNA. Translation: AAP91191.1.
AY339568 Genomic DNA. Translation: AAP91204.1.
AY339569 Genomic DNA. Translation: AAP91217.1.
AY339570 Genomic DNA. Translation: AAP91230.1.
AY339571 Genomic DNA. Translation: AAP91243.1.
AY339572 Genomic DNA. Translation: AAP91256.1.
AY339573 Genomic DNA. Translation: AAP91269.1.
AY339574 Genomic DNA. Translation: AAP91282.1.
AY339575 Genomic DNA. Translation: AAP91295.1.
AY339576 Genomic DNA. Translation: AAP91308.1.
AF346970 Genomic DNA. Translation: AAK17308.1.
AF346971 Genomic DNA. Translation: AAK17321.1.
AF346972 Genomic DNA. Translation: AAK17334.1.
AF346976 Genomic DNA. Translation: AAK17386.1.
AF346977 Genomic DNA. Translation: AAK17399.1.
AF346979 Genomic DNA. Translation: AAK17425.1.
AF346980 Genomic DNA. Translation: AAK17438.1.
AF346981 Genomic DNA. Translation: AAK17451.1.
AF346982 Genomic DNA. Translation: AAK17464.1.
AF346984 Genomic DNA. Translation: AAK17490.1.
AF346988 Genomic DNA. Translation: AAK17542.1.
AF346989 Genomic DNA. Translation: AAK17555.1.
AF346990 Genomic DNA. Translation: AAK17568.1.
AF346991 Genomic DNA. Translation: AAK17581.1.
AF346993 Genomic DNA. Translation: AAK17607.1.
AF346994 Genomic DNA. Translation: AAK17620.1.
AF347000 Genomic DNA. Translation: AAK17698.1.
AF347001 Genomic DNA. Translation: AAK17711.1.
AF347006 Genomic DNA. Translation: AAK17776.1.
AF347007 Genomic DNA. Translation: AAK17789.1.
AF347010 Genomic DNA. Translation: AAK17828.1.
AF347011 Genomic DNA. Translation: AAK17841.1.
AF347012 Genomic DNA. Translation: AAK17854.1.
AF347013 Genomic DNA. Translation: AAK17867.1.
AY289051 Genomic DNA. Translation: AAP47890.1.
AY289053 Genomic DNA. Translation: AAP47916.1.
AY289056 Genomic DNA. Translation: AAP47955.1.
AY289057 Genomic DNA. Translation: AAP47968.1.
AY289058 Genomic DNA. Translation: AAP47981.1.
AY289061 Genomic DNA. Translation: AAP48020.1.
AY289062 Genomic DNA. Translation: AAP48033.1.
AY289064 Genomic DNA. Translation: AAP48059.1.
AY289066 Genomic DNA. Translation: AAP48085.1.
AY289067 Genomic DNA. Translation: AAP48098.1.
AY289068 Genomic DNA. Translation: AAP48111.1.
AY289069 Genomic DNA. Translation: AAP48124.1.
AY289070 Genomic DNA. Translation: AAP48137.1.
AY289072 Genomic DNA. Translation: AAP48163.1.
AY289076 Genomic DNA. Translation: AAP48215.1.
AY289077 Genomic DNA. Translation: AAP48228.1.
AY289080 Genomic DNA. Translation: AAP48267.1.
AY289083 Genomic DNA. Translation: AAP48306.1.
AY289084 Genomic DNA. Translation: AAP48319.1.
AY289088 Genomic DNA. Translation: AAP48371.1.
AY289093 Genomic DNA. Translation: AAP48435.1.
AY289094 Genomic DNA. Translation: AAP48448.1.
AY289097 Genomic DNA. Translation: AAP48487.1.
AY289098 Genomic DNA. Translation: AAP48500.1.
AY289100 Genomic DNA. Translation: AAP48526.1.
AY289101 Genomic DNA. Translation: AAP48539.1.
AY289102 Genomic DNA. Translation: AAP48552.1.
AY495090 Genomic DNA. Translation: AAR92506.1.
AY495091 Genomic DNA. Translation: AAR92519.1.
AY495092 Genomic DNA. Translation: AAR92532.1.
AY495094 Genomic DNA. Translation: AAR92558.1.
AY495095 Genomic DNA. Translation: AAR92571.1.
AY495097 Genomic DNA. Translation: AAR92597.1.
AY495098 Genomic DNA. Translation: AAR92610.1.
AY495099 Genomic DNA. Translation: AAR92623.1.
AY495100 Genomic DNA. Translation: AAR92636.1.
AY495101 Genomic DNA. Translation: AAR92649.1.
AY495102 Genomic DNA. Translation: AAR92662.1.
AY495103 Genomic DNA. Translation: AAR92675.1.
AY495104 Genomic DNA. Translation: AAR92688.1.
AY495105 Genomic DNA. Translation: AAR92701.1.
AY495107 Genomic DNA. Translation: AAR92727.1.
AY495108 Genomic DNA. Translation: AAR92740.1.
AY495109 Genomic DNA. Translation: AAR92753.1.
AY495110 Genomic DNA. Translation: AAR92766.1.
AY495111 Genomic DNA. Translation: AAR92779.1.
AY495112 Genomic DNA. Translation: AAR92792.1.
AY495113 Genomic DNA. Translation: AAR92805.1.
AY495114 Genomic DNA. Translation: AAR92818.1.
AY495115 Genomic DNA. Translation: AAR92831.1.
AY495117 Genomic DNA. Translation: AAR92857.1.
AY495118 Genomic DNA. Translation: AAR92870.1.
AY495119 Genomic DNA. Translation: AAR92883.1.
AY495120 Genomic DNA. Translation: AAR92896.1.
AY495121 Genomic DNA. Translation: AAR92909.1.
AY495122 Genomic DNA. Translation: AAR92922.1.
AY495123 Genomic DNA. Translation: AAR92935.1.
AY495124 Genomic DNA. Translation: AAR92948.1.
AY495125 Genomic DNA. Translation: AAR92961.1.
AY495126 Genomic DNA. Translation: AAR92974.1.
AY495127 Genomic DNA. Translation: AAR92987.1.
AY495128 Genomic DNA. Translation: AAR93000.1.
AY495129 Genomic DNA. Translation: AAR93013.1.
AY495130 Genomic DNA. Translation: AAR93026.1.
AY495131 Genomic DNA. Translation: AAR93039.1.
AY495132 Genomic DNA. Translation: AAR93052.1.
AY495133 Genomic DNA. Translation: AAR93065.1.
AY495134 Genomic DNA. Translation: AAR93078.1.
AY495135 Genomic DNA. Translation: AAR93091.1.
AY495136 Genomic DNA. Translation: AAR93104.1.
AY495137 Genomic DNA. Translation: AAR93117.1.
AY495138 Genomic DNA. Translation: AAR93130.1.
AY495139 Genomic DNA. Translation: AAR93143.1.
AY495140 Genomic DNA. Translation: AAR93156.1.
AY495141 Genomic DNA. Translation: AAR93169.1.
AY495142 Genomic DNA. Translation: AAR93182.1.
AY495143 Genomic DNA. Translation: AAR93195.1.
AY495144 Genomic DNA. Translation: AAR93208.1.
AY495145 Genomic DNA. Translation: AAR93221.1.
AY495146 Genomic DNA. Translation: AAR93234.1.
AY495147 Genomic DNA. Translation: AAR93247.1.
AY495148 Genomic DNA. Translation: AAR93260.1.
AY495149 Genomic DNA. Translation: AAR93273.1.
AY495150 Genomic DNA. Translation: AAR93286.1.
AY495151 Genomic DNA. Translation: AAR93299.1.
AY495152 Genomic DNA. Translation: AAR93312.1.
AY495154 Genomic DNA. Translation: AAR93338.1.
AY495155 Genomic DNA. Translation: AAR93351.1.
AY495156 Genomic DNA. Translation: AAR93364.1.
AY495157 Genomic DNA. Translation: AAR93377.1.
AY495158 Genomic DNA. Translation: AAR93390.1.
AY495159 Genomic DNA. Translation: AAR93403.1.
AY495160 Genomic DNA. Translation: AAR93416.1.
AY495161 Genomic DNA. Translation: AAR93429.1.
AY495162 Genomic DNA. Translation: AAR93442.1.
AY495163 Genomic DNA. Translation: AAR93455.1.
AY495164 Genomic DNA. Translation: AAR93468.1.
AY495165 Genomic DNA. Translation: AAR93481.1.
AY495166 Genomic DNA. Translation: AAR93494.1.
AY495167 Genomic DNA. Translation: AAR93507.1.
AY495168 Genomic DNA. Translation: AAR93520.1.
AY495169 Genomic DNA. Translation: AAR93533.1.
AY495170 Genomic DNA. Translation: AAR93546.1.
AY495171 Genomic DNA. Translation: AAR93559.1.
AY495172 Genomic DNA. Translation: AAR93572.1.
AY495173 Genomic DNA. Translation: AAR93585.1.
AY495174 Genomic DNA. Translation: AAR93598.1.
AY495175 Genomic DNA. Translation: AAR93611.1.
AY495176 Genomic DNA. Translation: AAR93624.1.
AY495177 Genomic DNA. Translation: AAR93637.1.
AY495178 Genomic DNA. Translation: AAR93650.1.
AY495179 Genomic DNA. Translation: AAR93663.1.
AY495180 Genomic DNA. Translation: AAR93676.1.
AY495181 Genomic DNA. Translation: AAR93689.1.
AY495182 Genomic DNA. Translation: AAR93702.1.
AY495183 Genomic DNA. Translation: AAR93715.1.
AY495184 Genomic DNA. Translation: AAR93728.1.
AY495185 Genomic DNA. Translation: AAR93741.1.
AY495186 Genomic DNA. Translation: AAR93754.1.
AY495187 Genomic DNA. Translation: AAR93767.1.
AY495188 Genomic DNA. Translation: AAR93780.1.
AY495189 Genomic DNA. Translation: AAR93793.1.
AY495190 Genomic DNA. Translation: AAR93806.1.
AY495191 Genomic DNA. Translation: AAR93819.1.
AY495192 Genomic DNA. Translation: AAR93832.1.
AY495193 Genomic DNA. Translation: AAR93845.1.
AY495194 Genomic DNA. Translation: AAR93858.1.
AY495239 Genomic DNA. Translation: AAR94443.1.
AY495240 Genomic DNA. Translation: AAR94456.1.
AY495241 Genomic DNA. Translation: AAR94469.1.
AY495242 Genomic DNA. Translation: AAR94482.1.
AY495243 Genomic DNA. Translation: AAR94495.1.
AY495244 Genomic DNA. Translation: AAR94508.1.
AY495245 Genomic DNA. Translation: AAR94521.1.
AY495246 Genomic DNA. Translation: AAR94534.1.
AY495247 Genomic DNA. Translation: AAR94547.1.
AY495248 Genomic DNA. Translation: AAR94560.1.
AY495250 Genomic DNA. Translation: AAR94586.1.
AY495251 Genomic DNA. Translation: AAR94599.1.
AY495252 Genomic DNA. Translation: AAR94612.1.
AY495253 Genomic DNA. Translation: AAR94625.1.
AY495254 Genomic DNA. Translation: AAR94638.1.
AY495255 Genomic DNA. Translation: AAR94651.1.
AY495257 Genomic DNA. Translation: AAR94677.1.
AY495259 Genomic DNA. Translation: AAR94703.1.
AY495261 Genomic DNA. Translation: AAR94729.1.
AY495262 Genomic DNA. Translation: AAR94742.1.
AY495263 Genomic DNA. Translation: AAR94755.1.
AY495266 Genomic DNA. Translation: AAR94794.1.
AY495267 Genomic DNA. Translation: AAR94807.1.
AY495268 Genomic DNA. Translation: AAR94820.1.
AY495269 Genomic DNA. Translation: AAR94833.1.
AY495270 Genomic DNA. Translation: AAR94846.1.
AY495271 Genomic DNA. Translation: AAR94859.1.
AY495272 Genomic DNA. Translation: AAR94872.1.
AY495273 Genomic DNA. Translation: AAR94885.1.
AY495274 Genomic DNA. Translation: AAR94898.1.
AY495275 Genomic DNA. Translation: AAR94911.1.
AY495276 Genomic DNA. Translation: AAR94924.1.
AY495277 Genomic DNA. Translation: AAR94937.1.
AY495278 Genomic DNA. Translation: AAR94950.1.
AY495279 Genomic DNA. Translation: AAR94963.1.
AY495280 Genomic DNA. Translation: AAR94976.1.
AY495282 Genomic DNA. Translation: AAR95002.1.
AY495283 Genomic DNA. Translation: AAR95015.1.
AY495284 Genomic DNA. Translation: AAR95028.1.
AY495285 Genomic DNA. Translation: AAR95041.1.
AY495286 Genomic DNA. Translation: AAR95054.1.
AY495287 Genomic DNA. Translation: AAR95067.1.
AY495288 Genomic DNA. Translation: AAR95080.1.
AY495289 Genomic DNA. Translation: AAR95093.1.
AY495290 Genomic DNA. Translation: AAR95106.1.
AY495291 Genomic DNA. Translation: AAR95119.1.
AY495292 Genomic DNA. Translation: AAR95132.1.
AY495293 Genomic DNA. Translation: AAR95145.1.
AY495294 Genomic DNA. Translation: AAR95158.1.
AY495295 Genomic DNA. Translation: AAR95171.1.
AY495296 Genomic DNA. Translation: AAR95184.1.
AY495297 Genomic DNA. Translation: AAR95197.1.
AY495298 Genomic DNA. Translation: AAR95210.1.
AY495299 Genomic DNA. Translation: AAR95223.1.
AY495300 Genomic DNA. Translation: AAR95236.1.
AY495301 Genomic DNA. Translation: AAR95249.1.
AY495302 Genomic DNA. Translation: AAR95262.1.
AY495303 Genomic DNA. Translation: AAR95275.1.
AY495304 Genomic DNA. Translation: AAR95288.1.
AY495305 Genomic DNA. Translation: AAR95301.1.
AY495306 Genomic DNA. Translation: AAR95314.1.
AY495307 Genomic DNA. Translation: AAR95327.1.
AY495308 Genomic DNA. Translation: AAR95340.1.
AY495309 Genomic DNA. Translation: AAR95353.1.
AY495311 Genomic DNA. Translation: AAR95379.1.
AY495312 Genomic DNA. Translation: AAR95392.1.
AY495315 Genomic DNA. Translation: AAR95431.1.
AY495316 Genomic DNA. Translation: AAR95444.1.
AY495318 Genomic DNA. Translation: AAR95470.1.
AY495320 Genomic DNA. Translation: AAR95496.1.
AY495321 Genomic DNA. Translation: AAR95509.1.
AY495324 Genomic DNA. Translation: AAR95548.1.
AY495325 Genomic DNA. Translation: AAR95561.1.
AY495326 Genomic DNA. Translation: AAR95574.1.
AY495327 Genomic DNA. Translation: AAR95587.1.
AY495328 Genomic DNA. Translation: AAR95600.1.
AY495330 Genomic DNA. Translation: AAR95626.1.
PIRDNHUN5. A00446.
RefSeqYP_003024036.1. NC_012920.1.

3D structure databases

ProteinModelPortalP03915.
SMRP03915. Positions 1-602.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid110636. 3 interactions.
IntActP03915. 2 interactions.

Chemistry

BindingDBP03915.
ChEMBLCHEMBL2363065.
DrugBankDB00157. NADH.

PTM databases

PhosphoSiteP03915.

Polymorphism databases

DMDM6648059.

Proteomic databases

PaxDbP03915.
PRIDEP03915.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000361567; ENSP00000354813; ENSG00000198786.
GeneID4540.
KEGGhsa:4540.

Organism-specific databases

CTD4540.
GeneCardsGCMTP012339.
H-InvDBHIX0060142.
HGNCHGNC:7461. MT-ND5.
MIM252010. phenotype.
256000. phenotype.
516005. gene.
535000. phenotype.
540000. phenotype.
neXtProtNX_P03915.
Orphanet104. Leber hereditary optic neuropathy.
255210. Maternally-inherited Leigh syndrome.
550. MELAS syndrome.
551. MERRF syndrome.
255199. Sporadic Leigh syndrome.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG1009.
HOGENOMHOG000100570.
HOVERGENHBG108343.
InParanoidP03915.
KOK03883.
OMAKLDYFSM.
OrthoDBEOG75QR54.
PhylomeDBP03915.
ProtClustDBMTH00108.
TreeFamTF342974.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

ArrayExpressP03915.
BgeeP03915.
GenevestigatorP03915.

Family and domain databases

InterProIPR010934. NADH_DH_su5_C.
IPR001750. NADH_UbQ/plastoQ_OxRdtase.
IPR001516. NADH_UbQ_OxRdtase_chain5/L_N.
IPR003945. NADHpl_OxRdtase_5.
[Graphical view]
PfamPF06455. NADH5_C. 1 hit.
PF00361. Oxidored_q1. 1 hit.
PF00662. Oxidored_q1_N. 1 hit.
[Graphical view]
TIGRFAMsTIGR01974. NDH_I_L. 1 hit.
ProtoNetSearch...

Other

GenomeRNAi4540.
NextBio17511.
PROP03915.
SOURCESearch...

Entry information

Entry nameNU5M_HUMAN
AccessionPrimary (citable) accession number: P03915
Secondary accession number(s): Q34773, Q8WCY3
Entry history
Integrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: May 30, 2000
Last modified: April 16, 2014
This is version 145 of the entry and version 2 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