This gene encodes a subunit of the NADH:ubiquinone oxidoreductase (complex I), which is the first enzyme complex in the electron transport chain of mitochondria. This complex functions in the transfer of electrons from NADH to the respiratory chain. The subunit encoded by this gene is one of seven subunits in the iron-sulfur protein fraction. Mutations in this gene cause mitochondrial complex I deficiency, a disease that causes a wide variety of clinical disorders, including neonatal disease and adult-onset neurodegenerative disorders.[provided by RefSeq, Oct 2009]
Function:
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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.
Subcellular Location:
Mitochondrion inner membrane.
Similarity:
Belongs to the complex I NDUFS6 subunit family.
SWISS:
O75380
Gene ID:
4726
Database links:
Entrez Gene: 4726 Human
Entrez Gene: 407785 Mouse
Entrez Gene: 29478 Rat
Omim: 603848 Human
SwissProt: O75380 Human
SwissProt: P52503 Mouse
SwissProt: P52504 Rat
Unigene: 408257 Human
Unigene: 29897 Mouse
Unigene: 15883 Rat
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Sample:
Lane 1: Hela (Human) Cell Lysate at 30 ug
Lane 2: U937 (Human) Cell Lysate at 30 ug
Primary: Anti-NDUFS6 (SL19092R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 11 kD
Observed band size: 15 kD
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