InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: NDUFS1

Summary

InnateDB Protein

IDBP-388554.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

NDUFS1

Protein Name

NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)

Synonyms

CI-75k; CI-75Kd; PRO1304;

Species

Homo sapiens

Ensembl Protein

ENSP00000392709

InnateDB Gene

IDBG-79438 (NDUFS1)

Protein Structure

UniProt Annotation

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). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. {ECO:0000250}.

Subcellular Localization

Mitochondrion inner membrane.

Disease Associations

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. {ECO:0000269PubMed:11349233}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 47 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.

Experimentally validated
Total	47 [view]
Protein-Protein	44 [view]
Protein-DNA	3 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	1 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0005515	protein binding
GO:0008137	NADH dehydrogenase (ubiquinone) activity
GO:0009055	electron carrier activity
GO:0016491	oxidoreductase activity
GO:0016651	oxidoreductase activity, acting on NAD(P)H
GO:0046872	metal ion binding
GO:0051536	iron-sulfur cluster binding
GO:0051537	2 iron, 2 sulfur cluster binding
GO:0051539	4 iron, 4 sulfur cluster binding

Biological Process

GO:0006120	mitochondrial electron transport, NADH to ubiquinone
GO:0008637	apoptotic mitochondrial changes
GO:0022904	respiratory electron transport chain
GO:0042773	ATP synthesis coupled electron transport
GO:0044237	cellular metabolic process
GO:0044281	small molecule metabolic process
GO:0045333	cellular respiration
GO:0046034	ATP metabolic process
GO:0051881	regulation of mitochondrial membrane potential
GO:0055114	oxidation-reduction process
GO:0072593	reactive oxygen species metabolic process

Cellular Component

GO:0005739	mitochondrion
GO:0005743	mitochondrial inner membrane
GO:0005747	mitochondrial respiratory chain complex I
GO:0005758	mitochondrial intermembrane space
GO:0016020	membrane

Protein Structure and Domains

PDB ID

InterPro

IPR001041 2Fe-2S ferredoxin-type domain
IPR006656 Molybdopterin oxidoreductase
IPR010228 NADH:ubiquinone oxidoreductase, subunit G
IPR015405 NADH-quinone oxidoreductase, chain G, C-terminal
IPR019574 NADH:ubiquinone oxidoreductase, subunit G, iron-sulphur binding

PFAM

PF00111
PF00384
PF09326
PF10588

PRINTS

PIRSF

SMART

SM00929

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

P28331