Entity Details

Primary name NDUS1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP28331
EntryNameNDUS1_HUMAN
FullNameNADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length727
SequenceStatuscomplete
DateCreated1992-12-01
DateModified2021-06-02

Ontological Relatives

GenesNDUFS1

GO terms

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GOName
GO:0005739 mitochondrion
GO:0005747 mitochondrial respiratory chain complex I
GO:0005758 mitochondrial intermembrane space
GO:0005759 mitochondrial matrix
GO:0006120 mitochondrial electron transport, NADH to ubiquinone
GO:0008137 NADH dehydrogenase (ubiquinone) activity
GO:0008637 apoptotic mitochondrial changes
GO:0009055 electron transfer activity
GO:0032981 mitochondrial respiratory chain complex I assembly
GO:0045333 cellular respiration
GO:0046034 ATP metabolic process
GO:0046872 metal ion binding
GO:0051537 2 iron, 2 sulfur cluster binding
GO:0051539 4 iron, 4 sulfur cluster binding
GO:0051881 regulation of mitochondrial membrane potential
GO:0072593 reactive oxygen species metabolic process

Subcellular Location

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Subcellular Location
Mitochondrion inner membrane

Domains

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DomainNameCategoryType
IPR000283 NADH:ubiquinone oxidoreductase, 75kDa subunit, conserved siteSiteConserved site
IPR001041 2Fe-2S ferredoxin-type iron-sulfur binding domainDomainDomain
IPR006656 Molybdopterin oxidoreductaseDomainDomain
IPR006963 Molybdopterin oxidoreductase, 4Fe-4S domainDomainDomain
IPR010228 NADH:ubiquinone oxidoreductase, subunit GFamilyFamily
IPR015405 NADH-quinone oxidoreductase, chain G, C-terminalDomainDomain
IPR019574 NADH:ubiquinone oxidoreductase, subunit G, iron-sulphur bindingDomainDomain
IPR036010 2Fe-2S ferredoxin-like superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
618226 OMIMMitochondrial complex I deficiency, nuclear type 5 (MC1DN5)A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging 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. MC1DN5 transmission pattern is consistent with autosomal recessive inheritance. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00157 NADHDrugbanksmall molecule

Interactions

39 interactions

InteractorPartnerSourcesPublicationsLink
NDUS1_HUMANC1T9A_HUMANBioGRID, IntAct23414517 details
NDUS1_HUMANECE2_HUMANMINT23455924 details
NDUS1_HUMANEFMT4_HUMANMINT23455924 details
NDUS1_HUMANEFCE2_HUMANMINT23455924 details
NDUS1_HUMANHD_HUMANIntAct17500595 32814053 details
NDUS1_HUMANMET27_HUMANIntAct32814053 details
NDUS1_HUMANBOD1_HUMANIntAct32814053 details
NDUS1_HUMANTHAP3_HUMANIntAct32814053 details
NDUS1_HUMANCOG8_HUMANIntAct32814053 details
NDUS1_HUMANBRME1_HUMANIntAct32814053 details
NDUS1_HUMANLRC61_HUMANIntAct32814053 details
NDUS1_HUMANDPYL5_HUMANIntAct32814053 details
NDUS1_HUMANF135B_HUMANIntAct32814053 details
NDUS1_HUMANLAR4B_HUMANIntAct32814053 details
NDUS1_HUMANHAUS7_HUMANIntAct32814053 details
NDUS1_HUMANDELE1_HUMANIntAct32814053 details
NDUS1_HUMANK0408_HUMANIntAct32814053 details
NDUS1_HUMANPIMT_HUMANIntAct32814053 details
NDUS1_HUMANNDF1_HUMANIntAct32814053 details
NDUS1_HUMANCX7A1_HUMANIntAct32814053 details
NDUS1_HUMANKLF5_HUMANIntAct32814053 details
NDUS1_HUMANWFS1_HUMANBioGRID, IntAct31536960 32814053 details
NDUS1_HUMANKIF1B_HUMANIntAct32814053 details
NDUS1_HUMANCASP3_HUMANBioGRID15186778 details
NDUS1_HUMANKDM1A_HUMANBioGRID23455924 details
NDUS1_HUMANMDM2_HUMANBioGRID30879903 details
NDUS1_HUMANHLAB_HUMANIntAct17353931 details
NDUS1_HUMANNDUA9_HUMANBioGRID, IntAct, MINT22939629 24344204 26344197 27173435 31536960 unassigned1312 details
NDUS1_HUMANSTOM_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANPIGS_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANGPAA1_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANGDIA_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANSOAT1_HUMANBioGRID, IntAct27173435 29128334 unassigned1312 details
NDUS1_HUMANACAP2_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANEHBP1_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANMBOA7_HUMANBioGRID, IntAct27173435 unassigned1312 details
NDUS1_HUMANTNIK_HUMANIntAct31413325 details
NDUS1_HUMANOSMR_HUMANMINT32807793 details
NDUS1_HUMANMDHM_HUMANBioGRID, HPRD32877691 6439716 details