Q4P8D2 · MIA40_USTMA

Function

function

Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Forms a redox cycle with ERV1 that involves a disulfide relay system. Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS (By similarity).

Cofactor

Cu2+ (UniProtKB | Rhea| CHEBI:29036 )

Zn2+ (UniProtKB | Rhea| CHEBI:29105 )

Note: Cu2+ or Zn2+.

GO annotations

all annotationsall molecular functionvirus receptor activitydna bindingrna bindingcytoskeletal motor activitycatalytic activitygtpase activitystructural molecule activitytransporter activitycytoskeletal protein bindinglipid bindingcyclase activityantioxidant activityoxidoreductase activitytransferase activityhydrolase activitylyase activityisomerase activityligase activityprotein tag activitycargo receptor activityhistone bindingprotein folding chaperonetranslation regulator activitynutrient reservoir activityreceptor ligand activitymolecular transducer activitymolecular adaptor activitytoxin activitycell adhesion mediator activitymolecular function regulator activityvirus coreceptor activitycatalytic activity, acting on a proteincatalytic activity, acting on dnacatalytic activity, acting on rnamolecular carrier activitytranscription regulator activitygeneral transcription initiation factor activitymolecular sensor activitymolecular sequestering activityatp-dependent activityother molecular functionall biological processmitotic cell cyclecytokinesiscytoplasmic translationimmune system processmuscle system processcirculatory system processrenal system processrespiratory system processcarbohydrate metabolic processgeneration of precursor metabolites and energydna replicationdna repairdna recombinationchromatin organizationdna-templated transcriptionregulation of dna-templated transcriptiontrna metabolic processprotein foldingprotein glycosylationamino acid metabolic processmodified amino acid metabolic processlipid metabolic processvitamin metabolic processsulfur compound metabolic processintracellular protein transportnucleocytoplasmic transportautophagyinflammatory responsemitochondrion organizationcytoskeleton organizationmicrotubule-based movementperoxisome organizationlysosome organizationchromosome segregationcell adhesionestablishment or maintenance of cell polarityprogrammed cell deathphotosynthesismrna metabolic processsnrna metabolic processvesicle-mediated transportreproductive processdigestive system processsignalingcell differentiationprotein catabolic processextracellular matrix organizationregulatory ncrna-mediated gene silencingtelomere organizationcell junction organizationwound healingribosome biogenesiscilium organizationanatomical structure developmentcell motilitynervous system processendocrine processprotein maturationtransmembrane transportnucleobase-containing small molecule metabolic processhepaticobiliary system processmembrane organizationprotein-containing complex assemblycell wall organization or biogenesisnitrogen cycle metabolic processprotein localization to plasma membranedefense response to other organismdetoxificationmeiotic nuclear divisionmitotic nuclear divisionmitochondrial gene expressioncarbohydrate derivative metabolic processother biological processall cellular componentnuclear chromosomeextracellular regionextracellular spacecell wallnucleusnuclear envelopenucleoplasmchromosomenucleolusmitochondrionlysosomeendosomevacuoleperoxisomeendoplasmic reticulumgolgi apparatuslipid dropletmicrotubule organizing centercytosolribosomecytoskeletonplasma membraneciliumplastidthylakoidexternal encapsulating structureextracellular matrixcytoplasmic vesicleorganelleother cellular component
Cell color indicative of number of GO terms
AspectTerm
Cellular Componentmitochondrial inner membrane
Cellular Componentmitochondrial intermembrane space
Molecular Functionprotein-disulfide reductase activity
Biological Processprotein import into mitochondrial intermembrane space
Biological Processprotein maturation by protein folding

Keywords

Names & Taxonomy

Protein names

  • Recommended name
    Mitochondrial intermembrane space import and assembly protein 40
  • Alternative names
    • Mitochondrial import inner membrane translocase TIM40

Gene names

    • Name
      MIA40
    • Synonyms
      TIM40
    • ORF names
      UMAG_03631

Organism names

Accessions

  • Primary accession
    Q4P8D2
  • Secondary accessions
    • A0A0D1E1T3

Proteomes

Organism-specific databases

Subcellular Location

Features

Showing features for topological domain, transmembrane.

Type
IDPosition(s)Description
Topological domain29-45Mitochondrial matrix
Transmembrane46-63Helical; Signal-anchor for type II membrane protein
Topological domain64-247Mitochondrial intermembrane

Keywords

PTM/Processing

Features

Showing features for transit peptide, chain, disulfide bond.

Type
IDPosition(s)Description
Transit peptide1-28Mitochondrion
ChainPRO_000023529329-247Mitochondrial intermembrane space import and assembly protein 40
Disulfide bond149↔151Redox-active
Disulfide bond160↔193
Disulfide bond170↔183

Keywords

Interaction

Subunit

Monomer.

Protein-protein interaction databases

Structure

Family & Domains

Features

Showing features for region, domain, motif, compositional bias.

Type
IDPosition(s)Description
Region81-114Disordered
Domain157-201CHCH
Motif160-170Cx9C motif 1
Motif183-193Cx9C motif 2
Region217-247Disordered
Compositional bias218-232Polar residues

Domain

The CHCH domain contains a conserved twin Cys-X9-Cys motif which is required for import and stability of MIA40 in mitochondria.

Keywords

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Sequence processing
    The displayed sequence is further processed into a mature form.
  • Length
    247
  • Mass (Da)
    26,545
  • Last updated
    2005-07-19 v1
  • Checksum
    CABFAF16B884EC3C
MLSSKLVACSAGRSVQRISRTFLPAMRGVATKAAAGPSRQSALSSYSIAAVTAIGVGASFYALQSRSSAIQCEPRQAWHDRLKPKEAKGDATLHKDAHTRHAPAEVQDERVEPVEETPVAIEVAVEESEEQTGQQSAYDPETGEINWDCPCLGGMAHGPCGEQFKLAFSCFVYSEAEPKGIDCVDKFKAMQDCFREHPDVYKDEIEDDEAANAQFEKEEANAKSNGLNDAAQEAVEESSGGKEGASA

Features

Showing features for compositional bias.

TypeIDPosition(s)Description
Compositional bias218-232Polar residues

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
CM003148
EMBL· GenBank· DDBJ
KIS68545.1
EMBL· GenBank· DDBJ
Genomic DNA

Genome annotation databases

Similar Proteins

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