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