Proteins/UNIPROT:Q8N183

NDUFAF2

ProteinUNIPROT:Q8N183

Acts as a molecular chaperone for mitochondrial complex I assembly. 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. Is involved in the initial steps of cilia formation, including removal of CP110 from the mother centrioles, docking of membrane vesicles to the mother centrioles, and establishment of the transition zone..

Function from UniProtKB (Q8N183), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

4 rows

Leigh syndrome has a germline basis in NDUFAF2DiseaseGenCC HGNC:28086|MONDO:0009723Inferred only
mitochondrial complex I deficiency has a germline basis in NDUFAF2DiseaseGenCC HGNC:28086|MONDO:0100133Inferred only
mitochondrial complex I deficiency, nuclear type 10 has a germline basis in NDUFAF2DiseaseGenCC HGNC:28086|MONDO:0032616Inferred only
mitochondrial disease has a germline basis in NDUFAF2DiseaseGenCC HGNC:28086|MONDO:0044970Inferred only

Statements

5 statements

5 statements · 2 reference databases · Subject: NDUFAF2 · Sources: Hetionet, Reactome

Order

UBC interacts with NDUFAF2ProteinHetionet hetionet:interacts:7316-91942Inferred only
NDUFAF2 participates in Aerobic respiration and respiratory electron transportPathwayReactome R-HSA-1428517Inferred only
NDUFAF2 participates in Complex I biogenesisPathwayReactome R-HSA-6799198Inferred only
NDUFAF2 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
NDUFAF2 participates in Respiratory electron transportPathwayReactome R-HSA-611105Inferred only

Other names

7 names

Resolves to: NDUFAF2

Also called: B17.2-likeB17.2LMimitinMMTNMyc-induced mitochondrial proteinNDUFA12-like proteinNDUFA12L

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