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P03923 (NU6M_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 139. 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 6

EC=1.6.5.3
Alternative name(s):
NADH dehydrogenase subunit 6
Gene names
Name:MT-ND6
Synonyms:MTND6, NADH6, ND6
Encoded onMitochondrion
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length174 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 Potential.

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.4 Ref.11 Ref.14 Ref.15 Ref.16

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.12 Ref.13

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.17

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.18 Ref.19

Sequence similarities

Belongs to the complex I subunit 6 family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 174174NADH-ubiquinone oxidoreductase chain 6
PRO_0000118291

Regions

Transmembrane1 – 2121Helical; Potential
Transmembrane26 – 4621Helical; Potential
Transmembrane47 – 6721Helical; Potential
Transmembrane86 – 10621Helical; Potential
Transmembrane111 – 13121Helical; Potential
Transmembrane151 – 17121Helical; Potential

Natural variations

Natural variant261I → M in LDYT; possible rare primary mutation. Ref.13
VAR_008394
Natural variant311V → A. Ref.16
VAR_014393
Natural variant331I → V. Ref.16
VAR_014394
Natural variant361G → S in LHON; possible rare primary mutation. Ref.15
VAR_008395
Natural variant581I → V. Ref.9
VAR_014395
Natural variant591Y → C in LHON; possible rare primary mutation. Ref.14
VAR_008396
Natural variant601L → S in LHON; possible rare primary mutation. Ref.16
VAR_014396
Natural variant631M → V in MT-C1D. Ref.18 Ref.19
VAR_064568
Natural variant641M → I in LHON; possible rare primary mutation.
VAR_008512
Natural variant641M → V in LHON; primary mutation; low severity; up to 50% of vision recovery. Ref.4 Ref.11
VAR_004763
Natural variant721A → V in MT-C1D and LDYT; most severe mutation with no vision recovery. Ref.12 Ref.19
VAR_004764
Natural variant741A → V in MELAS. Ref.17
VAR_014397
Natural variant1651Y → C. Ref.10
VAR_008604

Experimental info

Sequence conflict1021L → F in CAA24037. Ref.1
Sequence conflict1341L → F in CAA24037. Ref.1
Sequence conflict1591T → P in CAA24037. Ref.1

Sequences

Sequence LengthMass (Da)Tools
P03923 [UniParc].

Last modified May 30, 2000. Version 2.
Checksum: E50CD9F3FAFC600D

FASTA17418,622
        10         20         30         40         50         60 
MMYALFLLSV GLVMGFVGFS SKPSPIYGGL VLIVSGVVGC VIILNFGGGY MGLMVFLIYL 

        70         80         90        100        110        120 
GGMMVVFGYT TAMAIEEYPE AWGSGVEVLV SVLVGLAMEV GLVLWVKEYD GVVVVVNFNS 

       130        140        150        160        170 
VGSWMIYEGE GSGLIREDPI GAGALYDYGR WLVVVTGWTL FVGVYIVIEI ARGN 

« 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 102; 134 AND 159.
[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].
Tissue: Placenta.
[4]"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: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT LHON VAL-64.
[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]"Patients with idiopathic cardiomyopathy belong to the same mitochondrial DNA gene family of Parkinson's disease and mitochondrial encephalomyopathy."
Ozawa T., Tanaka M., Sugiyama S., Ino H., Ohno K., Hattori K., Ohbayashi T., Ito T., Deguchi H., Kawamura K., Nakane Y., Hashiba K.
Biochem. Biophys. Res. Commun. 177:518-525(1991) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT VAL-58.
[10]"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: VARIANT CYS-165.
[11]"An ND-6 mitochondrial DNA mutation associated with Leber hereditary optic neuropathy."
Johns D.R., Neufeld M.J., Park R.D.
Biochem. Biophys. Res. Commun. 187:1551-1557(1992) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON VAL-64.
[12]"A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia."
Jun A.S., Brown M.D., Wallace D.C.
Proc. Natl. Acad. Sci. U.S.A. 91:6206-6210(1994) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LDYT VAL-72.
[13]"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 MET-26.
[14]"Leber's hereditary optic neuropathy: clinical and molecular genetic results obtained in a family with a new point mutation at nucleotide position 14498 in the ND 6 gene."
Leo-Kottler B., Christ-Adler M., Baumann B., Zrenner E., Wissinger B.
Ger. J. Ophthalmol. 5:233-240(1996) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON CYS-59.
[15]"Leber's hereditary optic neuropathy: clinical and molecular genetic findings in a patient with a new mutation in the ND6 gene."
Besch D., Leo-Kottler B., Zrenner E., Wissinger B.
Graefes Arch. Clin. Exp. Ophthalmol. 237:745-752(1999) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON SER-36.
[16]"The mitochondrial ND6 gene is a hot spot for mutations that cause Leber's hereditary optic neuropathy."
Chinnery P.F., Brown D.T., Andrews R.M., Singh-Kler R., Riordan-Eva P., Lindley J., Applegarth D.A., Turnbull D.M., Howell N.
Brain 124:209-218(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT LHON SER-60, VARIANTS ALA-31 AND VAL-33.
[17]"An mtDNA mutation, 14453G-->A, in the NADH dehydrogenase subunit 6 associated with severe MELAS syndrome."
Ravn K., Wibrand F., Hansen F.J., Horn N., Rosenberg T., Schwartz M.
Eur. J. Hum. Genet. 9:805-809(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT MELAS VAL-74.
[18]"Impaired complex I assembly in a Leigh syndrome patient with a novel missense mutation in the ND6 gene."
Ugalde C., Triepels R.H., Coenen M.J., van den Heuvel L.P., Smeets R., Uusimaa J., Briones P., Campistol J., Majamaa K., Smeitink J.A., Nijtmans L.G.
Ann. Neurol. 54:665-669(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT MT-C1D VAL-63.
[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: VARIANTS MT-C1D VAL-63 AND VAL-72.

Web resources

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
J01415 Genomic DNA. Translation: AAB58954.1.
V00662 Genomic DNA. Translation: CAA24037.1.
D38112 Genomic DNA. Translation: BAA07298.1.
AY063322 Genomic DNA. Translation: AAL47640.1.
AY339402 Genomic DNA. Translation: AAP89047.1.
AY339403 Genomic DNA. Translation: AAP89060.1.
AY339404 Genomic DNA. Translation: AAP89073.1.
AY339405 Genomic DNA. Translation: AAP89086.1.
AY339406 Genomic DNA. Translation: AAP89099.1.
AY339407 Genomic DNA. Translation: AAP89112.1.
AY339408 Genomic DNA. Translation: AAP89125.1.
AY339409 Genomic DNA. Translation: AAP89138.1.
AY339410 Genomic DNA. Translation: AAP89151.1.
AY339411 Genomic DNA. Translation: AAP89164.1.
AY339413 Genomic DNA. Translation: AAP89190.1.
AY339414 Genomic DNA. Translation: AAP89203.1.
AY339415 Genomic DNA. Translation: AAP89216.1.
AY339416 Genomic DNA. Translation: AAP89229.1.
AY339417 Genomic DNA. Translation: AAP89242.1.
AY339418 Genomic DNA. Translation: AAP89255.1.
AY339419 Genomic DNA. Translation: AAP89268.1.
AY339420 Genomic DNA. Translation: AAP89281.1.
AY339421 Genomic DNA. Translation: AAP89294.1.
AY339422 Genomic DNA. Translation: AAP89307.1.
AY339423 Genomic DNA. Translation: AAP89320.1.
AY339424 Genomic DNA. Translation: AAP89333.1.
AY339425 Genomic DNA. Translation: AAP89346.1.
AY339426 Genomic DNA. Translation: AAP89359.1.
AY339427 Genomic DNA. Translation: AAP89372.1.
AY339428 Genomic DNA. Translation: AAP89385.1.
AY339429 Genomic DNA. Translation: AAP89398.1.
AY339430 Genomic DNA. Translation: AAP89411.1.
AY339431 Genomic DNA. Translation: AAP89424.1.
AY339432 Genomic DNA. Translation: AAP89437.1.
AY339433 Genomic DNA. Translation: AAP89450.1.
AY339434 Genomic DNA. Translation: AAP89463.1.
AY339435 Genomic DNA. Translation: AAP89476.1.
AY339436 Genomic DNA. Translation: AAP89489.1.
AY339437 Genomic DNA. Translation: AAP89502.1.
AY339438 Genomic DNA. Translation: AAP89515.1.
AY339439 Genomic DNA. Translation: AAP89528.1.
AY339440 Genomic DNA. Translation: AAP89541.1.
AY339441 Genomic DNA. Translation: AAP89554.1.
AY339442 Genomic DNA. Translation: AAP89567.1.
AY339443 Genomic DNA. Translation: AAP89580.1.
AY339444 Genomic DNA. Translation: AAP89593.1.
AY339445 Genomic DNA. Translation: AAP89606.1.
AY339446 Genomic DNA. Translation: AAP89619.1.
AY339447 Genomic DNA. Translation: AAP89632.1.
AY339448 Genomic DNA. Translation: AAP89645.1.
AY339449 Genomic DNA. Translation: AAP89658.1.
AY339450 Genomic DNA. Translation: AAP89671.1.
AY339455 Genomic DNA. Translation: AAP89736.1.
AY339456 Genomic DNA. Translation: AAP89749.1.
AY339457 Genomic DNA. Translation: AAP89762.1.
AY339458 Genomic DNA. Translation: AAP89775.1.
AY339459 Genomic DNA. Translation: AAP89788.1.
AY339460 Genomic DNA. Translation: AAP89801.1.
AY339461 Genomic DNA. Translation: AAP89814.1.
AY339462 Genomic DNA. Translation: AAP89827.1.
AY339463 Genomic DNA. Translation: AAP89840.1.
AY339464 Genomic DNA. Translation: AAP89853.1.
AY339465 Genomic DNA. Translation: AAP89866.1.
AY339466 Genomic DNA. Translation: AAP89879.1.
AY339467 Genomic DNA. Translation: AAP89892.1.
AY339468 Genomic DNA. Translation: AAP89905.1.
AY339469 Genomic DNA. Translation: AAP89918.1.
AY339470 Genomic DNA. Translation: AAP89931.1.
AY339471 Genomic DNA. Translation: AAP89944.1.
AY339472 Genomic DNA. Translation: AAP89957.1.
AY339473 Genomic DNA. Translation: AAP89970.1.
AY339474 Genomic DNA. Translation: AAP89983.1.
AY339475 Genomic DNA. Translation: AAP89996.1.
AY339476 Genomic DNA. Translation: AAP90009.1.
AY339477 Genomic DNA. Translation: AAP90022.1.
AY339478 Genomic DNA. Translation: AAP90035.1.
AY339479 Genomic DNA. Translation: AAP90048.1.
AY339480 Genomic DNA. Translation: AAP90061.1.
AY339481 Genomic DNA. Translation: AAP90074.1.
AY339482 Genomic DNA. Translation: AAP90087.1.
AY339483 Genomic DNA. Translation: AAP90100.1.
AY339484 Genomic DNA. Translation: AAP90113.1.
AY339485 Genomic DNA. Translation: AAP90126.1.
AY339486 Genomic DNA. Translation: AAP90139.1.
AY339487 Genomic DNA. Translation: AAP90152.1.
AY339488 Genomic DNA. Translation: AAP90165.1.
AY339489 Genomic DNA. Translation: AAP90178.1.
AY339490 Genomic DNA. Translation: AAP90191.1.
AY339491 Genomic DNA. Translation: AAP90204.1.
AY339492 Genomic DNA. Translation: AAP90217.1.
AY339493 Genomic DNA. Translation: AAP90230.1.
AY339494 Genomic DNA. Translation: AAP90243.1.
AY339495 Genomic DNA. Translation: AAP90256.1.
AY339496 Genomic DNA. Translation: AAP90269.1.
AY339497 Genomic DNA. Translation: AAP90282.1.
AY339498 Genomic DNA. Translation: AAP90295.1.
AY339499 Genomic DNA. Translation: AAP90308.1.
AY339500 Genomic DNA. Translation: AAP90321.1.
AY339501 Genomic DNA. Translation: AAP90334.1.
AY339502 Genomic DNA. Translation: AAP90347.1.
AY339503 Genomic DNA. Translation: AAP90360.1.
AY339504 Genomic DNA. Translation: AAP90373.1.
AY339505 Genomic DNA. Translation: AAP90386.1.
AY339506 Genomic DNA. Translation: AAP90399.1.
AY339507 Genomic DNA. Translation: AAP90412.1.
AY339508 Genomic DNA. Translation: AAP90425.1.
AY339509 Genomic DNA. Translation: AAP90438.1.
AY339510 Genomic DNA. Translation: AAP90451.1.
AY339511 Genomic DNA. Translation: AAP90464.1.
AY339512 Genomic DNA. Translation: AAP90477.1.
AY339513 Genomic DNA. Translation: AAP90490.1.
AY339514 Genomic DNA. Translation: AAP90503.1.
AY339515 Genomic DNA. Translation: AAP90516.1.
AY339516 Genomic DNA. Translation: AAP90529.1.
AY339517 Genomic DNA. Translation: AAP90542.1.
AY339518 Genomic DNA. Translation: AAP90555.1.
AY339519 Genomic DNA. Translation: AAP90568.1.
AY339520 Genomic DNA. Translation: AAP90581.1.
AY339521 Genomic DNA. Translation: AAP90594.1.
AY339522 Genomic DNA. Translation: AAP90607.1.
AY339523 Genomic DNA. Translation: AAP90620.1.
AY339524 Genomic DNA. Translation: AAP90633.1.
AY339525 Genomic DNA. Translation: AAP90646.1.
AY339526 Genomic DNA. Translation: AAP90659.1.
AY339528 Genomic DNA. Translation: AAP90685.1.
AY339529 Genomic DNA. Translation: AAP90698.1.
AY339530 Genomic DNA. Translation: AAP90711.1.
AY339531 Genomic DNA. Translation: AAP90724.1.
AY339532 Genomic DNA. Translation: AAP90737.1.
AY339533 Genomic DNA. Translation: AAP90750.1.
AY339534 Genomic DNA. Translation: AAP90763.1.
AY339535 Genomic DNA. Translation: AAP90776.1.
AY339536 Genomic DNA. Translation: AAP90789.1.
AY339537 Genomic DNA. Translation: AAP90802.1.
AY339538 Genomic DNA. Translation: AAP90815.1.
AY339539 Genomic DNA. Translation: AAP90828.1.
AY339540 Genomic DNA. Translation: AAP90841.1.
AY339541 Genomic DNA. Translation: AAP90854.1.
AY339542 Genomic DNA. Translation: AAP90867.1.
AY339543 Genomic DNA. Translation: AAP90880.1.
AY339544 Genomic DNA. Translation: AAP90893.1.
AY339545 Genomic DNA. Translation: AAP90906.1.
AY339546 Genomic DNA. Translation: AAP90919.1.
AY339547 Genomic DNA. Translation: AAP90932.1.
AY339548 Genomic DNA. Translation: AAP90945.1.
AY339549 Genomic DNA. Translation: AAP90958.1.
AY339550 Genomic DNA. Translation: AAP90971.1.
AY339551 Genomic DNA. Translation: AAP90984.1.
AY339552 Genomic DNA. Translation: AAP90997.1.
AY339553 Genomic DNA. Translation: AAP91010.1.
AY339554 Genomic DNA. Translation: AAP91023.1.
AY339555 Genomic DNA. Translation: AAP91036.1.
AY339556 Genomic DNA. Translation: AAP91049.1.
AY339557 Genomic DNA. Translation: AAP91062.1.
AY339558 Genomic DNA. Translation: AAP91075.1.
AY339559 Genomic DNA. Translation: AAP91088.1.
AY339560 Genomic DNA. Translation: AAP91101.1.
AY339561 Genomic DNA. Translation: AAP91114.1.
AY339562 Genomic DNA. Translation: AAP91127.1.
AY339563 Genomic DNA. Translation: AAP91140.1.
AY339564 Genomic DNA. Translation: AAP91153.1.
AY339565 Genomic DNA. Translation: AAP91166.1.
AY339566 Genomic DNA. Translation: AAP91179.1.
AY339567 Genomic DNA. Translation: AAP91192.1.
AY339568 Genomic DNA. Translation: AAP91205.1.
AY339569 Genomic DNA. Translation: AAP91218.1.
AY339570 Genomic DNA. Translation: AAP91231.1.
AY339571 Genomic DNA. Translation: AAP91244.1.
AY339572 Genomic DNA. Translation: AAP91257.1.
AY339573 Genomic DNA. Translation: AAP91270.1.
AY339574 Genomic DNA. Translation: AAP91283.1.
AY339575 Genomic DNA. Translation: AAP91296.1.
AY339576 Genomic DNA. Translation: AAP91309.1.
AY339577 Genomic DNA. Translation: AAP91322.1.
AY339578 Genomic DNA. Translation: AAP91335.1.
AY339579 Genomic DNA. Translation: AAP91348.1.
AY339580 Genomic DNA. Translation: AAP91361.1.
AY339581 Genomic DNA. Translation: AAP91374.1.
AY339582 Genomic DNA. Translation: AAP91387.1.
AY339583 Genomic DNA. Translation: AAP91400.1.
AY339584 Genomic DNA. Translation: AAP91413.1.
AY339585 Genomic DNA. Translation: AAP91426.1.
AY339586 Genomic DNA. Translation: AAP91439.1.
AY339587 Genomic DNA. Translation: AAP91452.1.
AY339588 Genomic DNA. Translation: AAP91465.1.
AY339589 Genomic DNA. Translation: AAP91478.1.
AY339590 Genomic DNA. Translation: AAP91491.1.
AY339591 Genomic DNA. Translation: AAP91504.1.
AY339592 Genomic DNA. Translation: AAP91517.1.
AY339593 Genomic DNA. Translation: AAP91530.1.
AF346964 Genomic DNA. Translation: AAK17231.1.
AF346965 Genomic DNA. Translation: AAK17244.1.
AF346966 Genomic DNA. Translation: AAK17257.1.
AF346967 Genomic DNA. Translation: AAK17270.1.
AF346971 Genomic DNA. Translation: AAK17322.1.
AF346972 Genomic DNA. Translation: AAK17335.1.
AF346973 Genomic DNA. Translation: AAK17348.1.
AF346974 Genomic DNA. Translation: AAK17361.1.
AF346975 Genomic DNA. Translation: AAK17374.1.
AF346976 Genomic DNA. Translation: AAK17387.1.
AF346977 Genomic DNA. Translation: AAK17400.1.
AF346978 Genomic DNA. Translation: AAK17413.1.
AF346981 Genomic DNA. Translation: AAK17452.1.
AF346982 Genomic DNA. Translation: AAK17465.1.
AF346983 Genomic DNA. Translation: AAK17478.1.
AF346984 Genomic DNA. Translation: AAK17491.1.
AF346985 Genomic DNA. Translation: AAK17504.1.
AF346988 Genomic DNA. Translation: AAK17543.1.
AF346989 Genomic DNA. Translation: AAK17556.1.
AF346990 Genomic DNA. Translation: AAK17569.1.
AF346993 Genomic DNA. Translation: AAK17608.1.
AF346994 Genomic DNA. Translation: AAK17621.1.
AF346995 Genomic DNA. Translation: AAK17634.1.
AF346998 Genomic DNA. Translation: AAK17673.1.
AF346999 Genomic DNA. Translation: AAK17686.1.
AF347000 Genomic DNA. Translation: AAK17699.1.
AF347001 Genomic DNA. Translation: AAK17712.1.
AF347002 Genomic DNA. Translation: AAK17725.1.
AF347003 Genomic DNA. Translation: AAK17738.1.
AF347004 Genomic DNA. Translation: AAK17751.1.
AF347005 Genomic DNA. Translation: AAK17764.1.
AF347006 Genomic DNA. Translation: AAK17777.1.
AF347007 Genomic DNA. Translation: AAK17790.1.
AF347008 Genomic DNA. Translation: AAK17803.1.
AF347009 Genomic DNA. Translation: AAK17816.1.
AF347010 Genomic DNA. Translation: AAK17829.1.
AF347014 Genomic DNA. Translation: AAK17881.1.
AF347015 Genomic DNA. Translation: AAK17894.1.
AY289052 Genomic DNA. Translation: AAP47904.1.
AY289053 Genomic DNA. Translation: AAP47917.1.
AY289056 Genomic DNA. Translation: AAP47956.1.
AY289057 Genomic DNA. Translation: AAP47969.1.
AY289058 Genomic DNA. Translation: AAP47982.1.
AY289059 Genomic DNA. Translation: AAP47995.1.
AY289060 Genomic DNA. Translation: AAP48008.1.
AY289062 Genomic DNA. Translation: AAP48034.1.
AY289063 Genomic DNA. Translation: AAP48047.1.
AY289064 Genomic DNA. Translation: AAP48060.1.
AY289065 Genomic DNA. Translation: AAP48073.1.
AY289066 Genomic DNA. Translation: AAP48086.1.
AY289067 Genomic DNA. Translation: AAP48099.1.
AY289068 Genomic DNA. Translation: AAP48112.1.
AY289069 Genomic DNA. Translation: AAP48125.1.
AY289070 Genomic DNA. Translation: AAP48138.1.
AY289071 Genomic DNA. Translation: AAP48151.1.
AY289072 Genomic DNA. Translation: AAP48164.1.
AY289073 Genomic DNA. Translation: AAP48177.1.
AY289074 Genomic DNA. Translation: AAP48190.1.
AY289075 Genomic DNA. Translation: AAP48203.1.
AY289076 Genomic DNA. Translation: AAP48216.1.
AY289077 Genomic DNA. Translation: AAP48229.1.
AY289078 Genomic DNA. Translation: AAP48242.1.
AY289079 Genomic DNA. Translation: AAP48255.1.
AY289080 Genomic DNA. Translation: AAP48268.1.
AY289081 Genomic DNA. Translation: AAP48281.1.
AY289082 Genomic DNA. Translation: AAP48294.1.
AY289083 Genomic DNA. Translation: AAP48307.1.
AY289084 Genomic DNA. Translation: AAP48320.1.
AY289085 Genomic DNA. Translation: AAP48333.1.
AY289086 Genomic DNA. Translation: AAP48346.1.
AY289088 Genomic DNA. Translation: AAP48372.1.
AY289089 Genomic DNA. Translation: AAP48385.1.
AY289090 Genomic DNA. Translation: AAP48398.1.
AY289091 Genomic DNA. Translation: AAP48411.1.
AY289092 Genomic DNA. Translation: AAP48423.1.
AY289093 Genomic DNA. Translation: AAP48436.1.
AY289094 Genomic DNA. Translation: AAP48449.1.
AY289095 Genomic DNA. Translation: AAP48462.1.
AY289096 Genomic DNA. Translation: AAP48475.1.
AY289097 Genomic DNA. Translation: AAP48488.1.
AY289098 Genomic DNA. Translation: AAP48501.1.
AY289099 Genomic DNA. Translation: AAP48514.1.
AY289100 Genomic DNA. Translation: AAP48527.1.
AY289101 Genomic DNA. Translation: AAP48540.1.
AY289102 Genomic DNA. Translation: AAP48553.1.
AY495090 Genomic DNA. Translation: AAR92507.1.
AY495091 Genomic DNA. Translation: AAR92520.1.
AY495092 Genomic DNA. Translation: AAR92533.1.
AY495093 Genomic DNA. Translation: AAR92546.1.
AY495094 Genomic DNA. Translation: AAR92559.1.
AY495096 Genomic DNA. Translation: AAR92585.1.
AY495097 Genomic DNA. Translation: AAR92598.1.
AY495098 Genomic DNA. Translation: AAR92611.1.
AY495099 Genomic DNA. Translation: AAR92624.1.
AY495100 Genomic DNA. Translation: AAR92637.1.
AY495101 Genomic DNA. Translation: AAR92650.1.
AY495102 Genomic DNA. Translation: AAR92663.1.
AY495103 Genomic DNA. Translation: AAR92676.1.
AY495104 Genomic DNA. Translation: AAR92689.1.
AY495105 Genomic DNA. Translation: AAR92702.1.
AY495106 Genomic DNA. Translation: AAR92715.1.
AY495107 Genomic DNA. Translation: AAR92728.1.
AY495109 Genomic DNA. Translation: AAR92754.1.
AY495110 Genomic DNA. Translation: AAR92767.1.
AY495112 Genomic DNA. Translation: AAR92793.1.
AY495113 Genomic DNA. Translation: AAR92806.1.
AY495114 Genomic DNA. Translation: AAR92819.1.
AY495115 Genomic DNA. Translation: AAR92832.1.
AY495116 Genomic DNA. Translation: AAR92845.1.
AY495117 Genomic DNA. Translation: AAR92858.1.
AY495118 Genomic DNA. Translation: AAR92871.1.
AY495119 Genomic DNA. Translation: AAR92884.1.
AY495120 Genomic DNA. Translation: AAR92897.1.
AY495121 Genomic DNA. Translation: AAR92910.1.
AY495122 Genomic DNA. Translation: AAR92923.1.
AY495124 Genomic DNA. Translation: AAR92949.1.
AY495125 Genomic DNA. Translation: AAR92962.1.
AY495126 Genomic DNA. Translation: AAR92975.1.
AY495128 Genomic DNA. Translation: AAR93001.1.
AY495129 Genomic DNA. Translation: AAR93014.1.
AY495130 Genomic DNA. Translation: AAR93027.1.
AY495131 Genomic DNA. Translation: AAR93040.1.
AY495132 Genomic DNA. Translation: AAR93053.1.
AY495133 Genomic DNA. Translation: AAR93066.1.
AY495134 Genomic DNA. Translation: AAR93079.1.
AY495135 Genomic DNA. Translation: AAR93092.1.
AY495136 Genomic DNA. Translation: AAR93105.1.
AY495137 Genomic DNA. Translation: AAR93118.1.
AY495138 Genomic DNA. Translation: AAR93131.1.
AY495139 Genomic DNA. Translation: AAR93144.1.
AY495140 Genomic DNA. Translation: AAR93157.1.
AY495141 Genomic DNA. Translation: AAR93170.1.
AY495142 Genomic DNA. Translation: AAR93183.1.
AY495143 Genomic DNA. Translation: AAR93196.1.
AY495144 Genomic DNA. Translation: AAR93209.1.
AY495145 Genomic DNA. Translation: AAR93222.1.
AY495146 Genomic DNA. Translation: AAR93235.1.
AY495147 Genomic DNA. Translation: AAR93248.1.
AY495148 Genomic DNA. Translation: AAR93261.1.
AY495149 Genomic DNA. Translation: AAR93274.1.
AY495150 Genomic DNA. Translation: AAR93287.1.
AY495151 Genomic DNA. Translation: AAR93300.1.
AY495152 Genomic DNA. Translation: AAR93313.1.
AY495153 Genomic DNA. Translation: AAR93326.1.
AY495154 Genomic DNA. Translation: AAR93339.1.
AY495155 Genomic DNA. Translation: AAR93352.1.
AY495156 Genomic DNA. Translation: AAR93365.1.
AY495157 Genomic DNA. Translation: AAR93378.1.
AY495158 Genomic DNA. Translation: AAR93391.1.
AY495159 Genomic DNA. Translation: AAR93404.1.
AY495160 Genomic DNA. Translation: AAR93417.1.
AY495161 Genomic DNA. Translation: AAR93430.1.
AY495162 Genomic DNA. Translation: AAR93443.1.
AY495163 Genomic DNA. Translation: AAR93456.1.
AY495164 Genomic DNA. Translation: AAR93469.1.
AY495165 Genomic DNA. Translation: AAR93482.1.
AY495166 Genomic DNA. Translation: AAR93495.1.
AY495167 Genomic DNA. Translation: AAR93508.1.
AY495168 Genomic DNA. Translation: AAR93521.1.
AY495169 Genomic DNA. Translation: AAR93534.1.
AY495170 Genomic DNA. Translation: AAR93547.1.
AY495171 Genomic DNA. Translation: AAR93560.1.
AY495172 Genomic DNA. Translation: AAR93573.1.
AY495173 Genomic DNA. Translation: AAR93586.1.
AY495174 Genomic DNA. Translation: AAR93599.1.
AY495175 Genomic DNA. Translation: AAR93612.1.
AY495176 Genomic DNA. Translation: AAR93625.1.
AY495177 Genomic DNA. Translation: AAR93638.1.
AY495180 Genomic DNA. Translation: AAR93677.1.
AY495185 Genomic DNA. Translation: AAR93742.1.
AY495187 Genomic DNA. Translation: AAR93768.1.
AY495188 Genomic DNA. Translation: AAR93781.1.
AY495189 Genomic DNA. Translation: AAR93794.1.
AY495190 Genomic DNA. Translation: AAR93807.1.
AY495191 Genomic DNA. Translation: AAR93820.1.
AY495192 Genomic DNA. Translation: AAR93833.1.
AY495193 Genomic DNA. Translation: AAR93846.1.
AY495194 Genomic DNA. Translation: AAR93859.1.
AY495195 Genomic DNA. Translation: AAR93872.1.
AY495196 Genomic DNA. Translation: AAR93885.1.
AY495197 Genomic DNA. Translation: AAR93898.1.
AY495198 Genomic DNA. Translation: AAR93911.1.
AY495199 Genomic DNA. Translation: AAR93924.1.
AY495200 Genomic DNA. Translation: AAR93937.1.
AY495201 Genomic DNA. Translation: AAR93950.1.
AY495202 Genomic DNA. Translation: AAR93963.1.
AY495203 Genomic DNA. Translation: AAR93976.1.
AY495204 Genomic DNA. Translation: AAR93989.1.
AY495205 Genomic DNA. Translation: AAR94002.1.
AY495206 Genomic DNA. Translation: AAR94015.1.
AY495207 Genomic DNA. Translation: AAR94028.1.
AY495208 Genomic DNA. Translation: AAR94041.1.
AY495210 Genomic DNA. Translation: AAR94067.1.
AY495211 Genomic DNA. Translation: AAR94080.1.
AY495212 Genomic DNA. Translation: AAR94093.1.
AY495213 Genomic DNA. Translation: AAR94106.1.
AY495214 Genomic DNA. Translation: AAR94119.1.
AY495216 Genomic DNA. Translation: AAR94145.1.
AY495217 Genomic DNA. Translation: AAR94158.1.
AY495218 Genomic DNA. Translation: AAR94171.1.
AY495219 Genomic DNA. Translation: AAR94184.1.
AY495220 Genomic DNA. Translation: AAR94197.1.
AY495221 Genomic DNA. Translation: AAR94210.1.
AY495222 Genomic DNA. Translation: AAR94223.1.
AY495223 Genomic DNA. Translation: AAR94236.1.
AY495224 Genomic DNA. Translation: AAR94249.1.
AY495225 Genomic DNA. Translation: AAR94262.1.
AY495226 Genomic DNA. Translation: AAR94275.1.
AY495227 Genomic DNA. Translation: AAR94288.1.
AY495228 Genomic DNA. Translation: AAR94301.1.
AY495229 Genomic DNA. Translation: AAR94314.1.
AY495230 Genomic DNA. Translation: AAR94327.1.
AY495231 Genomic DNA. Translation: AAR94340.1.
AY495232 Genomic DNA. Translation: AAR94353.1.
AY495233 Genomic DNA. Translation: AAR94366.1.
AY495234 Genomic DNA. Translation: AAR94379.1.
AY495235 Genomic DNA. Translation: AAR94392.1.
AY495236 Genomic DNA. Translation: AAR94405.1.
AY495237 Genomic DNA. Translation: AAR94418.1.
AY495238 Genomic DNA. Translation: AAR94431.1.
AY495239 Genomic DNA. Translation: AAR94444.1.
AY495240 Genomic DNA. Translation: AAR94457.1.
AY495241 Genomic DNA. Translation: AAR94470.1.
AY495242 Genomic DNA. Translation: AAR94483.1.
AY495243 Genomic DNA. Translation: AAR94496.1.
AY495244 Genomic DNA. Translation: AAR94509.1.
AY495245 Genomic DNA. Translation: AAR94522.1.
AY495246 Genomic DNA. Translation: AAR94535.1.
AY495247 Genomic DNA. Translation: AAR94548.1.
AY495248 Genomic DNA. Translation: AAR94561.1.
AY495249 Genomic DNA. Translation: AAR94574.1.
AY495250 Genomic DNA. Translation: AAR94587.1.
AY495251 Genomic DNA. Translation: AAR94600.1.
AY495252 Genomic DNA. Translation: AAR94613.1.
AY495253 Genomic DNA. Translation: AAR94626.1.
AY495254 Genomic DNA. Translation: AAR94639.1.
AY495255 Genomic DNA. Translation: AAR94652.1.
AY495256 Genomic DNA. Translation: AAR94665.1.
AY495257 Genomic DNA. Translation: AAR94678.1.
AY495258 Genomic DNA. Translation: AAR94691.1.
AY495259 Genomic DNA. Translation: AAR94704.1.
AY495261 Genomic DNA. Translation: AAR94730.1.
AY495262 Genomic DNA. Translation: AAR94743.1.
AY495263 Genomic DNA. Translation: AAR94756.1.
AY495264 Genomic DNA. Translation: AAR94769.1.
AY495266 Genomic DNA. Translation: AAR94795.1.
AY495267 Genomic DNA. Translation: AAR94808.1.
AY495268 Genomic DNA. Translation: AAR94821.1.
AY495269 Genomic DNA. Translation: AAR94834.1.
AY495270 Genomic DNA. Translation: AAR94847.1.
AY495271 Genomic DNA. Translation: AAR94860.1.
AY495272 Genomic DNA. Translation: AAR94873.1.
AY495273 Genomic DNA. Translation: AAR94886.1.
AY495274 Genomic DNA. Translation: AAR94899.1.
AY495275 Genomic DNA. Translation: AAR94912.1.
AY495276 Genomic DNA. Translation: AAR94925.1.
AY495277 Genomic DNA. Translation: AAR94938.1.
AY495278 Genomic DNA. Translation: AAR94951.1.
AY495279 Genomic DNA. Translation: AAR94964.1.
AY495280 Genomic DNA. Translation: AAR94977.1.
AY495281 Genomic DNA. Translation: AAR94990.1.
AY495282 Genomic DNA. Translation: AAR95003.1.
AY495283 Genomic DNA. Translation: AAR95016.1.
AY495284 Genomic DNA. Translation: AAR95029.1.
AY495285 Genomic DNA. Translation: AAR95042.1.
AY495286 Genomic DNA. Translation: AAR95055.1.
AY495287 Genomic DNA. Translation: AAR95068.1.
AY495288 Genomic DNA. Translation: AAR95081.1.
AY495289 Genomic DNA. Translation: AAR95094.1.
AY495290 Genomic DNA. Translation: AAR95107.1.
AY495291 Genomic DNA. Translation: AAR95120.1.
AY495292 Genomic DNA. Translation: AAR95133.1.
AY495293 Genomic DNA. Translation: AAR95146.1.
AY495294 Genomic DNA. Translation: AAR95159.1.
AY495295 Genomic DNA. Translation: AAR95172.1.
AY495296 Genomic DNA. Translation: AAR95185.1.
AY495297 Genomic DNA. Translation: AAR95198.1.
AY495298 Genomic DNA. Translation: AAR95211.1.
AY495299 Genomic DNA. Translation: AAR95224.1.
AY495300 Genomic DNA. Translation: AAR95237.1.
AY495301 Genomic DNA. Translation: AAR95250.1.
AY495302 Genomic DNA. Translation: AAR95263.1.
AY495303 Genomic DNA. Translation: AAR95276.1.
AY495304 Genomic DNA. Translation: AAR95289.1.
AY495305 Genomic DNA. Translation: AAR95302.1.
AY495306 Genomic DNA. Translation: AAR95315.1.
AY495307 Genomic DNA. Translation: AAR95328.1.
AY495308 Genomic DNA. Translation: AAR95341.1.
AY495310 Genomic DNA. Translation: AAR95367.1.
AY495311 Genomic DNA. Translation: AAR95380.1.
AY495312 Genomic DNA. Translation: AAR95393.1.
AY495313 Genomic DNA. Translation: AAR95406.1.
AY495314 Genomic DNA. Translation: AAR95419.1.
AY495315 Genomic DNA. Translation: AAR95432.1.
AY495316 Genomic DNA. Translation: AAR95445.1.
AY495317 Genomic DNA. Translation: AAR95458.1.
AY495318 Genomic DNA. Translation: AAR95471.1.
AY495319 Genomic DNA. Translation: AAR95484.1.
AY495320 Genomic DNA. Translation: AAR95497.1.
AY495321 Genomic DNA. Translation: AAR95510.1.
AY495322 Genomic DNA. Translation: AAR95523.1.
AY495323 Genomic DNA. Translation: AAR95536.1.
AY495324 Genomic DNA. Translation: AAR95549.1.
AY495325 Genomic DNA. Translation: AAR95562.1.
AY495326 Genomic DNA. Translation: AAR95575.1.
AY495327 Genomic DNA. Translation: AAR95588.1.
AY495328 Genomic DNA. Translation: AAR95601.1.
AY495329 Genomic DNA. Translation: AAR95614.1.
AY495330 Genomic DNA. Translation: AAR95627.1.
PIRDEHUN6. A00455.
RefSeqYP_003024037.1. NC_012920.1.

3D structure databases

ProteinModelPortalP03923.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid110637. 2 interactions.
IntActP03923. 1 interaction.

Chemistry

BindingDBP03923.
ChEMBLCHEMBL2363065.
DrugBankDB00157. NADH.

PTM databases

PhosphoSiteP03923.

Polymorphism databases

DMDM6648060.

Proteomic databases

PaxDbP03923.
PRIDEP03923.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000361681; ENSP00000354665; ENSG00000198695.
GeneID4541.
KEGGhsa:4541.

Organism-specific databases

CTD4541.
GeneCardsGCMTM014151.
HGNCHGNC:7462. MT-ND6.
MIM252010. phenotype.
500001. phenotype.
516006. gene.
535000. phenotype.
540000. phenotype.
neXtProtNX_P03923.
Orphanet99718. Leber 'plus' disease.
104. Leber hereditary optic neuropathy.
255210. Maternally-inherited Leigh syndrome.
550. MELAS syndrome.
255199. Sporadic Leigh syndrome.
PharmGKBPA31266.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG70008.
HOVERGENHBG007487.
InParanoidP03923.
KOK03884.
OMAVVFGYTT.
OrthoDBEOG7HF1MX.
PhylomeDBP03923.
ProtClustDBMTH00109.
TreeFamTF343324.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

BgeeP03923.
GenevestigatorP03923.

Family and domain databases

InterProIPR001457. NADH_UbQ/plastoQ_OxRdtase_su6.
[Graphical view]
PfamPF00499. Oxidored_q3. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiMT-ND6.
GenomeRNAi4541.
NextBio17514.
PROP03923.
SOURCESearch...

Entry information

Entry nameNU6M_HUMAN
AccessionPrimary (citable) accession number: P03923
Secondary accession number(s): Q34774, Q8HG30
Entry history
Integrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: May 30, 2000
Last modified: April 16, 2014
This is version 139 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