Names & Taxonomy

Uniprot ID:
P28331
Entry Name:
NDUS1_HUMAN
Status:
reviewed
Protein Names:
NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-75kD) (CI-75kD)
Gene Names:
NDUFS1
Gene Names Primary:
NDUFS1
Organism:
Homo sapiens (Human)

Structure

Length:
727
Sequence:
MLRIPVRKALVGLSKSPKGCVRTTATAASNLIEVFVDGQSVMVEPGTTVLQACEKVGMQIPRFCYHERLSVAGNCRMCLVEIEKAPKVVAACAMPVMKGWNILTNSEKSKKAREGVMEFLLANHPLDCPICDQGGECDLQDQSMMFGNDRSRFLEGKRAVEDKNIGPLVKTIMTRCIQCTRCIRFASEIAGVDDLGTTGRGNDMQVGTYIEKMFMSELSGNIIDICPVGALTSKPYAFTARPWETRKTESIDVMDAVGSNIVVSTRTGEVMRILPRMHEDINEEWISDKTRFAYDGLKRQRLTEPMVRNEKGLLTYTSWEDALSRVAGMLQSFQGKDVAAIAGGLVDAEALVALKDLLNRVDSDTLCTEEVFPTAGAGTDLRSNYLLNTTIAGVEEADVVLLVGTNPRFEAPLFNARIRKSWLHNDLKVALIGSPVDLTYTYDHLGDSPKILQDIASGSHPFSQVLKEAKKPMVVLGSSALQRNDGAAILAAVSSIAQKIRMTSGVTGDWKVMNILHRIASQVAALDLGYKPGVEAIRKNPPKVLFLLGADGGCITRQDLPKDCFIIYQGHHGDVGAPIADVILPGAAYTEKSATYVNTEGRAQQTKVAVTPPGLAREDWKIIRALSEIAGMTLPYDTLDQVRNRLEEVSPNLVRYDDIEGANYFQQANELSKLVNQQLLADPLVPPQLTIKDFYMTDSISRASQTMAKCVKAVTEGAQAVEEPSIC
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion inner membrane.

Function

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.
Catalytic Activity:
NADH + ubiquinone + 5 H(+)(In) = NAD(+) + ubiquinol + 4 H(+)(Out).; NADH + acceptor = NAD(+) + reduced acceptor.
Cofactor:
COFACTOR: Name= cluster; Xref=ChEBI:CHEBI:49601;
Gene Ontology Go:
mitochondrial intermembrane space
mitochondrial matrix
mitochondrial respiratory chain complex I
mitochondrion
myelin sheath
2 iron, 2 sulfur cluster binding
4 iron, 4 sulfur cluster binding
electron carrier activity
iron-sulfur cluster binding
metal ion binding
NADH dehydrogenase (ubiquinone) activity
apoptotic mitochondrial changes
ATP metabolic process
cellular metabolic process
cellular respiration
mitochondrial electron transport, NADH to ubiquinone
mitochondrial respiratory chain complex I assembly
reactive oxygen species metabolic process
regulation of mitochondrial membrane potential
respiratory electron transport chain
small molecule metabolic process
Gene Ontology Biological Process:
apoptotic mitochondrial changes
ATP metabolic process
cellular metabolic process
cellular respiration
mitochondrial electron transport, NADH to ubiquinone
mitochondrial respiratory chain complex I assembly
reactive oxygen species metabolic process
regulation of mitochondrial membrane potential
respiratory electron transport chain
small molecule metabolic process
Gene Ontology Molecular Function:
2 iron, 2 sulfur cluster binding
4 iron, 4 sulfur cluster binding
electron carrier activity
iron-sulfur cluster binding
metal ion binding
NADH dehydrogenase (ubiquinone) activity
Gene Ontology Cellular Component:
mitochondrial intermembrane space
mitochondrial matrix
mitochondrial respiratory chain complex I
mitochondrion
myelin sheath
Keywords:
2Fe-2S
4Fe-4S
Acetylation
Alternative splicing
Complete proteome
Direct protein sequencing
Disease mutation
Electron transport
Iron
Iron-sulfur
Membrane
Metal-binding
Mitochondrion
Mitochondrion inner membrane
NAD
Oxidoreductase
Polymorphism
Reference proteome
Respiratory chain
Transit peptide
Transport
Ubiquinone

Publication

PubMed ID:
1935949 14702039 15815621 15489334 12611891 21269460 24275569 25944712 11349233 22499341