MIA40 / YKL195W Overview


Standard Name
MIA40 1
Systematic Name
YKL195W
SGD ID
SGD:S000001678
Aliases
TIM40 7 , FMP15
Feature Type
ORF , Verified
Description
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 1 2 3 4 5 6
Name Description
Mitochondrial intermembrane space Import and Assembly 1
Comparative Info
Sequence Details

Sequence

The S. cerevisiae Reference Genome sequence is derived from laboratory strain S288C. Download DNA or protein sequence, view genomic context and coordinates. Click "Sequence Details" to view all sequence information for this locus, including that for other strains.


Summary
MIA40/YKL195W is located on the left arm of chromosome XI between YKT6 v-SNARE and MST1 threonyl-tRNA synthetase; coding sequence is 1212 nucleotides long with 9 SNPs, 5 of which cause amino acid polymorphisms
Protein Details

Protein

Basic sequence-derived (length, molecular weight, isoelectric point) and experimentally-determined (median abundance, median absolute deviation) protein information. Click "Protein Details" for further information about the protein such as half-life, abundance, domains, domains shared with other proteins, protein sequence retrieval for various strains, physico-chemical properties, protein modification sites, and external identifiers for the protein.


Summary
Mia40p is 403 amino acids long, shorter-lived, low in abundance; contains disordered regions throughout the protein; succinylated on K333, sumoylated on K393 and K394, N-glycosylated on 3 asparagines, phosphorylated on 9 residues
Length (a.a.)
403
Mol. Weight (Da)
44491.8
Isoelectric Point
4.19
Median Abundance (molecules/cell)
4025 +/- 2248
Half-life (hr)
8.3

Alleles

Curated mutant alleles for the specified gene, listed alphabetically. Click on the allele name to open the allele page. Click "SGD search" to view all alleles in search results.


View all MIA40 alleles in SGD search

Gene Ontology Details

Gene Ontology

GO Annotations consist of four mandatory components: a gene product, a term from one of the three Gene Ontology (GO) controlled vocabularies (Molecular Function, Biological Process, and Cellular Component), a reference, and an evidence code. SGD has manually curated and high-throughput GO Annotations, both derived from the literature, as well as computational, or predicted, annotations. Click "Gene Ontology Details" to view all GO information and evidence for this locus as well as biological processes it shares with other genes.


Summary
Oxidoreductase involved in protein folding and protein transport into mitochondrial intermembrane space; localizes to mitochondria

View computational annotations

Molecular Function

Manually Curated

Biological Process

Manually Curated

Cellular Component

Manually Curated
Phenotype Details

Phenotype

Phenotype annotations for a gene are curated single mutant phenotypes that require an observable (e.g., "cell shape"), a qualifier (e.g., "abnormal"), a mutant type (e.g., null), strain background, and a reference. In addition, annotations are classified as classical genetics or high-throughput (e.g., large scale survey, systematic mutation set). Whenever possible, allele information and additional details are provided. Click "Phenotype Details" to view all phenotype annotations and evidence for this locus as well as phenotypes it shares with other genes.


Summary
MIA40/YKL195W is an essential gene; null mutants are inviable. Repressible mutants exhibit decreased mitochondrial transport and abnormal protein/peptide distribution. Reduction of function mutants show decreased competitive fitness. Conditional mutants display increased heat sensitivity and decreased protein/peptide accumulation. Overexpression of MIA40 results in abnormal cell cycle progression and decreased vegetative growth.
Disease Details

Disease

Disease Annotations consist of three mandatory components: a gene product, a term from the Disease Ontology (DO) controlled vocabulary and an evidence code. SGD provides manually curated DO Annotations derived from the literature. Click "Disease Details" to view all Disease information and evidence for this locus as well as diseases it shares with other genes.


Summary
Yeast MIA40 is homologous to human CHCHD4, and has been used to study dysfunctional biogenesis of mitochondrial respiratory chain complex I
Interaction Details

Interaction

Interaction annotations are curated by BioGRID and include physical or genetic interactions observed between at least two genes. An interaction annotation is composed of the interaction type, name of the interactor, assay type (e.g., Two-Hybrid), annotation type (e.g., manual or high-throughput), and a reference, as well as other experimental details. Click "Interaction Details" to view all interaction annotations and evidence for this locus, including an interaction visualization.


Summary
Mia40p interacts physically with proteins involved in protein targeting; MIA40 interacts genetically with genes involved in transcription

290 total interactions for 219 unique genes

Physical Interactions

  • Affinity Capture-MS: 23
  • Affinity Capture-RNA: 10
  • Affinity Capture-Western: 28
  • Biochemical Activity: 8
  • Co-fractionation: 1
  • Co-purification: 16
  • Protein-peptide: 1
  • Proximity Label-MS: 2
  • Reconstituted Complex: 27

Genetic Interactions

  • Dosage Growth Defect: 1
  • Dosage Rescue: 4
  • Negative Genetic: 90
  • Phenotypic Enhancement: 1
  • Phenotypic Suppression: 2
  • Positive Genetic: 74
  • Synthetic Lethality: 2
Regulation Details

Regulation

The number of putative Regulators (genes that regulate it) and Targets (genes it regulates) for the given locus, based on experimental evidence. This evidence includes data generated through high-throughput techniques. Click "Regulation Details" to view all regulation annotations, shared GO enrichment among regulation Targets, and a regulator/target diagram for the locus.


Summary
MIA40 promoter is bound by Med6p, Spt3p, and Xbp1p in response to heat; MIA40 promoter is bound by Fkh1p and Fkh2p in response to starvation
Regulators
8
Targets
0
Expression Details

Expression

Expression data are derived from records contained in the Gene Expression Omnibus (GEO), and are first log2 transformed and normalized. Referenced datasets may contain one or more condition(s), and as a result there may be a greater number of conditions than datasets represented in a single clickable histogram bar. The histogram division at 0.0 separates the down-regulated (green) conditions and datasets from those that are up-regulated (red). Click "Expression Details" to view all expression annotations and details for this locus, including a visualization of genes that share a similar expression pattern.


Summary Paragraph

A summary of the locus, written by SGD Biocurators following a thorough review of the literature. Links to gene names and curated GO terms are included within the Summary Paragraphs.


Last Updated: 2024-11-19

Literature Details

Literature

All manually curated literature for the specified gene, organized into topics according to their relevance to the gene (Primary Literature, Additional Literature, or Review). Click "Literature Details" to view all literature information for this locus, including shared literature between genes.


Primary
65
Additional
59
Reviews
50

Resources