NDUFV1 - NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial - human protein (Function)
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NDUFV1 »  NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial   [ EC ]
Protein also known as:  NADH-ubiquinone oxidoreductase 51 kDa subunit.
Gene name:  NDUFV1
Entry whose protein(s) existence is based on evidence at protein level
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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).  
  • CuratedUniProtKB
GO molecular function 
4 iron, 4 sulfur cluster bindingdefinition[GO:0051539]  
  • IEAUniProtKB KW
FMN bindingdefinition[GO:0010181] silver  
  • IEAInterPro 2 GO
Metal ion bindingdefinition[GO:0046872]  
  • IEAUniProtKB KW
NAD bindingdefinition[GO:0051287] silver  
  • IEAInterPro 2 GO
NADH dehydrogenase (ubiquinone) activitydefinition[GO:0008137]  
  • NASUniProtKB
GO biological process 
Mitochondrial electron transport, NADH to ubiquinonedefinition[GO:0006120]  
  • ICUniProtKB
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reactions
EC NADH + ubiquinone + 5 H(+)(In) NAD(+) + ubiquinol + 4 H(+)(Out).  
  • CuratedUniProtKB
EC NADH + acceptor NAD(+) + reduced acceptor.  
  • CuratedUniProtKB
It requires the following cofactors
Binds 1 FMN (Potential).  
  • CuratedUniProtKB
Binds 1 4Fe-4S cluster (Potential).  
  • CuratedUniProtKB
According to KEGG, this protein belongs to the following pathways:
Alzheimer's disease  hsa05010+4723  
Huntington's disease  hsa05016+4723  
Metabolic pathways  hsa01100+4723  
Oxidative phosphorylation  hsa00190+4723  
Parkinson's disease  hsa05012+4723  
According to Reactome, this protein belongs to the following pathway:
Respiratory electron transport  REACT_22393  


Biological process 
Electron transport  definition   [KW-0249]
Respiratory chain  definition   [KW-0679]
Transport  definition   [KW-0813]
Molecular function 
Oxidoreductase  definition   [KW-0560]
Technical term 
Reference proteome  definition   [KW-1185]

Further external links

GeneWiki: NDUFV1
GenomeRNAi: 4723
PRO: PR:P49821