Complex I intermediate-associated protein 30, mitochondrial

Function

As part of the MCIA complex, involved in the assembly of the mitochondrial complex I.

Classification

Family (Pfam)

PF08547 CIA30

InterPro

Galactose-bd-like_sf, NADH-UbQ_OxRdtase-assoc_prot30, NDUFAF1

Functional cluster

Phosphoribosyltransferases & related

Gene Ontology · 8

• GO:0160295 mitochondrial complex I intermediate assembly complex
• GO:0005743 mitochondrial inner membrane
• GO:0005759 mitochondrial matrix
• GO:0005739 mitochondrion
• GO:0051082 unfolded protein binding
• GO:0006120 mitochondrial electron transport, NADH to ubiquinone
• GO:0032981 mitochondrial respiratory chain complex I assembly
• GO:0065003 protein-containing complex assembly

Disease associations

• mitochondrial complex I deficiency, nuclear type 11 MONDO:0032617

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• Complex I intermediate-associated protein 30, mitochondrial 1.00
• Complex I intermediate-associated protein 30, mitochondrial 0.99
• Complex I intermediate-associated protein 30, mitochondrial 0.99
• Complex I intermediate-associated protein 30, mitochondrial 0.96
• Complex I intermediate-associated protein 30, mitochondrial 0.82
• Probable complex I intermediate-associated protein 30, mitochondrial 0.77
• Probable complex I intermediate-associated protein 30, mitochondrial 0.77
• Probable complex I intermediate-associated protein 30 0.71
• Complex I intermediate-associated protein 30, mitochondrial 0.56
• Uncharacterized protein C9E9.15 0.44
• Large ribosomal subunit protein mL45 0.41
• Small ribosomal subunit protein mS25 0.40

Co-cited proteins · studied together in the literature

• Evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial 4 shared papers
• Complex I assembly factor ACAD9, mitochondrial 3 shared papers
• Complex I assembly factor TMEM126B, mitochondrial 2 shared papers
• Transmembrane protein 186 1 shared papers
• Transmembrane protein 242 1 shared papers
• Cytochrome c oxidase assembly factor 1 homolog 1 shared papers
• NADH-ubiquinone oxidoreductase chain 3 2 shared papers
• Complex I assembly factor TIMMDC1, mitochondrial 1 shared papers
• ATP synthase F(0) complex subunit C3, mitochondrial 1 shared papers
• NADH dehydrogenase [ubiquinone] 1 subunit C2 1 shared papers
• Transmembrane protein 70, mitochondrial 1 shared papers
• NADH-ubiquinone oxidoreductase chain 2 1 shared papers

Literature · 13 cited papers

• TMEM70 and TMEM242 help to assemble the rotor ring of human ATP synthase and interact with assembly factors for complex I. Proc. Natl. Acad. Sci. U.S.A. · 2021
• Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I. Cell Rep. · 2020
• 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
• Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy. J. Med. Genet. · 2011
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I. Cell Metab. · 2010
• Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease. EMBO J. · 2007
• Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly. Genes Dev. · 2007
• Human mitochondrial complex I assembly is mediated by NDUFAF1. FEBS J. · 2005
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
• CIA30 complex I assembly factor: a candidate for human complex I deficiency? Hum. Genet. · 2002
• … and 1 more in the literature graph