NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3

Function

Essential factor for the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I).

Classification

Family (Pfam)

PF04430 DUF498

InterPro

MTH938-like_sf, NDUF3, NDUFAF3/AAMDC

Functional cluster

Membrane Channels & Lipid-Anchored Proteins

Gene Ontology · 4

• GO:0005743 mitochondrial inner membrane
• GO:0005739 mitochondrion
• GO:0005634 nucleus
• GO:0032981 mitochondrial respiratory chain complex I assembly

Disease associations

• mitochondrial complex I deficiency, nuclear type 18 MONDO:0032623

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 0.98
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 0.97
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 0.93
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 0.84
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 0.80
• Transmembrane protein 186 0.63
• Small ribosomal subunit protein uS3m 0.62
• Distal membrane-arm assembly complex protein 2 0.62
• Small ribosomal subunit protein uS2m 0.61
• Haloacid dehalogenase-like hydrolase domain-containing 5 0.61
• Small ribosomal subunit protein uS3m 0.60
• Haloacid dehalogenase-like hydrolase domain-containing 5 0.59

Co-cited proteins · studied together in the literature

• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 1 shared papers
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 1 shared papers
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial 1 shared papers

Literature · 6 cited papers

• Mutations in mitochondrial complex I assembly factor NDUFAF3 cause Leigh syndrome. Mol. Genet. Metab. · 2017
• N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
• An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease. Am. J. Hum. Genet. · 2009
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004