Proteins/UNIPROT:P28331

NDUFS1

ProteinUNIPROT:P28331

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for catalysing the entry and efficient transfer of electrons within complex I. Plays a key role in the assembly and stability of complex I and participates in the association of complex I with ubiquinol-cytochrome reductase complex (Complex III) to form supercomplexes..

Function from UniProtKB (P28331), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

4 rows

Leigh syndrome has a germline basis in NDUFS1DiseaseGenCC HGNC:7707|MONDO:0009723Inferred only
mitochondrial complex I deficiency has a germline basis in NDUFS1DiseaseGenCC HGNC:7707|MONDO:0100133Inferred only
mitochondrial complex I deficiency, nuclear type 5 has a germline basis in NDUFS1DiseaseGenCC HGNC:7707|MONDO:0032610Inferred only
mitochondrial disease has a germline basis in NDUFS1DiseaseGenCC HGNC:7707|MONDO:0044970Inferred only

Statements

48 statements

48 statements · 6 reference databases · Subject: NDUFS1 · Sources: Hetionet, Human-GEM, Reactome, Rhea, UniProt, WikiPathways

Order

NDUFS1 interacts with C1QTNF9ProteinHetionet hetionet:interacts:338872-4719Inferred only
CASP3 interacts with NDUFS1ProteinHetionet hetionet:interacts:4719-836Inferred only
NDUFS1 interacts with CUL3ProteinHetionet hetionet:interacts:4719-8452Inferred only
MDH2 interacts with NDUFS1ProteinHetionet hetionet:interacts:4191-4719Inferred only
MRPL58 interacts with NDUFS1ProteinHetionet hetionet:interacts:3396-4719Inferred only
MYC interacts with NDUFS1ProteinHetionet hetionet:interacts:4609-4719Inferred only
NDUFS1 interacts with SIRT7ProteinHetionet hetionet:interacts:4719-51547Inferred only
NDUFS1 interacts with SUMO2ProteinHetionet hetionet:interacts:4719-6613Inferred only
NDUFS1 interacts with UBCProteinHetionet hetionet:interacts:4719-7316Inferred only
NDUFS1 interacts with VCAM1ProteinHetionet hetionet:interacts:4719-7412Inferred only
NDUFS1 participates in Aerobic respiration and respiratory electron transportPathwayReactome R-HSA-1428517Inferred only
NDUFS1 participates in Complex I biogenesisPathwayReactome R-HSA-6799198Inferred only

Show the remaining 36

NDUFS1 participates in Electron transport chain OXPHOS system in mitochondriaPathwayWikiPathways WP111Inferred only
NDUFS1 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
NDUFS1 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
NDUFS1 participates in Mitochondrial complex I assembly model OXPHOS systemPathwayWikiPathways WP4324Inferred only
NDUFS1 participates in Mitochondrial protein degradationPathwayReactome R-HSA-9837999Inferred only
NDUFS1 participates in Nonalcoholic fatty liver diseasePathwayWikiPathways WP4396Inferred only
NDUFS1 participates in Oxidative phosphorylationPathwayWikiPathways WP623Inferred only
NDUFS1 participates in Respiratory electron transportPathwayReactome R-HSA-611105Inferred only
ABHD2 increases the expression of NDUFS1ProteinHetionet hetionet:regulates:11057-4719Inferred only
AQP12A increases the expression of NDUFS1ProteinHetionet hetionet:regulates:375318-4719Inferred only
B4GALT4 decreases the expression of NDUFS1ProteinHetionet hetionet:regulates:8702-4719Inferred only
NDUFS1 decreases the expression of BNIP3ProteinHetionet hetionet:regulates:4719-664Inferred only
NDUFS1 decreases the expression of BNIP3LProteinHetionet hetionet:regulates:4719-665Inferred only
NDUFS1 decreases the expression of CDC20ProteinHetionet hetionet:regulates:4719-991Inferred only
NDUFS1 decreases the expression of CDK7ProteinHetionet hetionet:regulates:4719-1022Inferred only
DECR1 decreases the expression of NDUFS1ProteinHetionet hetionet:regulates:1666-4719Inferred only
NDUFS1 decreases the expression of DERAProteinHetionet hetionet:regulates:4719-51071Inferred only
DNMT1 decreases the expression of NDUFS1ProteinHetionet hetionet:regulates:1786-4719Inferred only
NDUFS1 decreases the expression of DPYSL2ProteinHetionet hetionet:regulates:4719-1808Inferred only
EPOR increases the expression of NDUFS1ProteinHetionet hetionet:regulates:2057-4719Inferred only
GLI3 decreases the expression of NDUFS1ProteinHetionet hetionet:regulates:2737-4719Inferred only
NDUFS1 increases the expression of HERC6ProteinHetionet hetionet:regulates:4719-55008Inferred only
NDUFS1 increases the expression of HOOK2ProteinHetionet hetionet:regulates:4719-29911Inferred only
NDUFS1 decreases the expression of PCMT1ProteinHetionet hetionet:regulates:4719-5110Inferred only
NDUFS1 decreases the expression of PEG10ProteinHetionet hetionet:regulates:4719-23089Inferred only
NDUFS1 decreases the expression of PSME2ProteinHetionet hetionet:regulates:4719-5721Inferred only
NDUFS1 decreases the expression of RBM34ProteinHetionet hetionet:regulates:4719-23029Inferred only
NDUFS1 increases the expression of RGS2ProteinHetionet hetionet:regulates:4719-5997Inferred only
SLC35A1 decreases the expression of NDUFS1ProteinHetionet hetionet:regulates:10559-4719Inferred only
NDUFS1 decreases the expression of TUBA1AProteinHetionet hetionet:regulates:4719-7846Inferred only
NDUFS1 decreases the expression of TYMSProteinHetionet hetionet:regulates:4719-7298Inferred only
NDUFS1 requires the cofactor di-mu-sulfido-diironCompoundUniProt P28331Inferred only
NDUFS1 requires the cofactor tetra-mu3-sulfido-tetraironCompoundUniProt P28331Inferred only
hydron is transported by NDUFS1CompoundRhea RHEA:29091Inferred only
proton is transported by NDUFS1CompoundHuman-GEM MAR06921Inferred only
proton is transported by NDUFS1CompoundHuman-GEM MAR06500Inferred only

Reactions

3 reactions

MAM01329c+5proton+NADH(2-)MAM01328c+4proton+NAD(+)
Transported by NDUFS8 & COXFA4 & NDUFAB1 & NDUFA1 & NDUFS4 & NDUFB5 & NDUFB3 & NDUFC1 & NDUFA2 & NDUFS5 & NDUFS7 & NDUFS2 & NDUFS6 & NDUFB1 &

Other names

2 names

Resolves to: NDUFS1

Also called: CI-75kDComplex I-75kD

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