FEATURES

FAMILY

DESCRIPTION

Also known as NDUS1_MOUSE, Ndufs1. Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:27799543, PubMed:32072193). Essential for catalysing the entry and efficient transfer of electrons within complex I (PubMed:27799543). Plays a key role in the assembly and stability of complex I and participates in the association of complex I with ubiquinol-cytochrome reductase complex (Complex III) to form supercomplexes (PubMed:27799543). Core subunit of respiratory chain NADH dehydrogenase (Complex I) which is composed of 45 different subunits (By similarity). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme (By similarity). Complex I associates with ubiquinol-cytochrome reductase complex (Complex III) to form supercomplexes (PubMed:27799543). In astrocytes, less complex I is assembled into supercomplexes as compared to neurons (PubMed:27799543). Interacts with MDM2 (By similarity). Interacts with AKAP1 (PubMed:32072193).

EXTERNAL LINKS

Ndufs1

Mutations

Mutation(s) distribution

Mutations table