MIA40 Mia40p [Saccharomyces cerevisiae S288C] - Gene

Summary

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Official Symbol: MIA40
Official Full Name: Mia40p
Primary source: SGD:S000001678
Locus tag: YKL195W
See related: AllianceGenome:SGD:S000001678; FungiDB:YKL195W; VEuPathDB:YKL195W
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Saccharomyces cerevisiae S288C (strain: S288C)
Lineage: Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Also known as: FMP15; TIM40
Summary: Enables 2 iron, 2 sulfur cluster binding activity; protein disulfide isomerase activity; and thiol oxidase activity. Involved in protein folding and protein import into mitochondrial intermembrane space. Located in mitochondrial inner membrane. Is active in mitochondrial intermembrane space. Used to study mitochondrial complex I deficiency. Orthologous to human CHCHD4 (coiled-coil-helix-coiled-coil-helix domain containing 4). [provided by Alliance of Genome Resources, Nov 2024]
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Genomic context

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See MIA40 in Genome Data Viewer

Location:: chromosome: XI

Exon count:: 1

Sequence:: Chromosome: XI; NC_001143.9 (75821..77032)

Chromosome XI - NC_001143.9 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_001143.9

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Increased levels of mitochondrial import factor Mia40 prevent the aggregation of polyQ proteins in the cytosol.
Title: Increased levels of mitochondrial import factor Mia40 prevent the aggregation of polyQ proteins in the cytosol.
Redox characterisation of Erv1, a key component for protein import and folding in yeast mitochondria.
Title: Redox characterisation of Erv1, a key component for protein import and folding in yeast mitochondria.
AIF meets the CHCHD4/Mia40-dependent mitochondrial import pathway.
Title: AIF meets the CHCHD4/Mia40-dependent mitochondrial import pathway.
the question whether parasite Erv homologs might also exert the function of Mia40 was addressed in another set of complementation assays. However, neither the KISS domain nor other truncated or mutant LtErv constructs were able to rescue yeast cells that lack ScMia40
Title: A single-cysteine mutant and chimeras of essential Leishmania Erv can complement the loss of Erv1 but not of Mia40 in yeast.
Plant Erv1, unlike its homologs in fungi and animals, can promote protein import and oxidative protein folding in the IMS independently of the oxidoreductase Mia40.
Title: Erv1 of Arabidopsis thaliana can directly oxidize mitochondrial intermembrane space proteins in the absence of redox-active Mia40.
The authors show that the substrate-binding domain of Mia40 is both necessary and sufficient to promote protein import, indicating that trapping by Mia40 drives protein translocation.
Title: Mia40 is a trans-site receptor that drives protein import into the mitochondrial intermembrane space by hydrophobic substrate binding.
these results confirm that GSH is critical for cytosolic Fe-S protein biogenesis and iron regulation, whereas ruling out significant roles for Erv1 or Mia40 in these pathways.
Title: Cytosolic Fe-S Cluster Protein Maturation and Iron Regulation Are Independent of the Mitochondrial Erv1/Mia40 Import System.
Mia40 catalyses the oxidative folding of disulphide-containing proteins in the mitochondria.
Title: Mia40 targets cysteines in a hydrophobic environment to direct oxidative protein folding in the mitochondria.
Mia40 serves as an electron sink in the Mia40-Erv1 protein import pathway.
Title: Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway.
Mia40 role in mitochondrial oxidative protein folding and import
Title: Mia40 is optimized for function in mitochondrial oxidative protein folding and import.

Submit: New GeneRIF Correction See all GeneRIFs (34)

Pathways from PubChem

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Homology

Orthologs from OrthoDB

Gene Ontology Provided by SGD

Function	Evidence Code	Pubs
enables 2 iron, 2 sulfur cluster binding	IDA Inferred from Direct Assay more info	PubMed
enables oxidoreductase activity	IEA Inferred from Electronic Annotation more info
enables oxidoreductase activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables protein disulfide isomerase activity	IDA Inferred from Direct Assay more info	PubMed
enables protein-disulfide reductase activity	IBA Inferred from Biological aspect of Ancestor more info
enables protein-disulfide reductase activity	IEA Inferred from Electronic Annotation more info
enables thiol oxidase activity	IDA Inferred from Direct Assay more info	PubMed

Process	Evidence Code	Pubs
involved_in protein folding	IDA Inferred from Direct Assay more info	PubMed
involved_in protein import into mitochondrial intermembrane space	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein import into mitochondrial intermembrane space	IEA Inferred from Electronic Annotation more info
involved_in protein import into mitochondrial intermembrane space	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in protein import into mitochondrial intermembrane space	IPI Inferred from Physical Interaction more info	PubMed
involved_in protein maturation by protein folding	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein transport	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in mitochondrial inner membrane	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial inner membrane	IEA Inferred from Electronic Annotation more info
is_active_in mitochondrial intermembrane space	IBA Inferred from Biological aspect of Ancestor more info
is_active_in mitochondrial intermembrane space	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial intermembrane space	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: Mia40p

NP_012726.2

Import and assembly protein in mitochondrial intermembrane space; component of MIA pathway which mediates import and oxidative folding of substrates including small proteins containing twin cysteine motifs; acts in concert with Erv1p, which oxidizes the cysteine residues of Mia40p to comprise a disulfide relay system that catalyzes import; also mediates folding of Atp23p via a chaperone-like activity; forms a dimer that binds iron-sulfur cluster in vitro

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

NC_001143.9 Reference assembly

Range

75821..77032

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_001179761.1 → NP_012726.2 TPA: Mia40p [Saccharomyces cerevisiae S288C]

See identical proteins and their annotated locations for NP_012726.2

Status: REVIEWED

UniProtKB/Swiss-Prot

D6VX05, P36046

UniProtKB/TrEMBL

A6ZZD0, B3LQR0, C7GJ14, C8ZBY1, G2WHJ7

Conserved Domains (2) summary

COG5271
Location:78 → 248

MDN1; Midasin, AAA ATPase with vWA domain, involved in ribosome maturation [Translation, ribosomal structure and biogenesis]

pfam06747
Location:307 → 343

CHCH; CHCH domain