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Mitochondrial intermembrane space import and assembly protein 40 (Mitochondrial import inner membrane translocase TIM40)

 MIA40_KLULA             Reviewed;         406 AA.
Q6CSA1;
16-MAY-2006, integrated into UniProtKB/Swiss-Prot.
16-AUG-2004, sequence version 1.
10-OCT-2018, entry version 68.
RecName: Full=Mitochondrial intermembrane space import and assembly protein 40;
AltName: Full=Mitochondrial import inner membrane translocase TIM40;
Flags: Precursor;
Name=MIA40; Synonyms=TIM40; OrderedLocusNames=KLLA0D02706g;
Kluyveromyces lactis (strain ATCC 8585 / CBS 2359 / DSM 70799 / NBRC
1267 / NRRL Y-1140 / WM37) (Yeast) (Candida sphaerica).
Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina;
Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Kluyveromyces.
NCBI_TaxID=284590;
[1]
NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
STRAIN=ATCC 8585 / CBS 2359 / DSM 70799 / NBRC 1267 / NRRL Y-1140 /
WM37;
PubMed=15229592; DOI=10.1038/nature02579;
Dujon B., Sherman D., Fischer G., Durrens P., Casaregola S.,
Lafontaine I., de Montigny J., Marck C., Neuveglise C., Talla E.,
Goffard N., Frangeul L., Aigle M., Anthouard V., Babour A., Barbe V.,
Barnay S., Blanchin S., Beckerich J.-M., Beyne E., Bleykasten C.,
Boisrame A., Boyer J., Cattolico L., Confanioleri F., de Daruvar A.,
Despons L., Fabre E., Fairhead C., Ferry-Dumazet H., Groppi A.,
Hantraye F., Hennequin C., Jauniaux N., Joyet P., Kachouri R.,
Kerrest A., Koszul R., Lemaire M., Lesur I., Ma L., Muller H.,
Nicaud J.-M., Nikolski M., Oztas S., Ozier-Kalogeropoulos O.,
Pellenz S., Potier S., Richard G.-F., Straub M.-L., Suleau A.,
Swennen D., Tekaia F., Wesolowski-Louvel M., Westhof E., Wirth B.,
Zeniou-Meyer M., Zivanovic Y., Bolotin-Fukuhara M., Thierry A.,
Bouchier C., Caudron B., Scarpelli C., Gaillardin C., Weissenbach J.,
Wincker P., Souciet J.-L.;
"Genome evolution in yeasts.";
Nature 430:35-44(2004).
-!- 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). {ECO:0000250}.
-!- COFACTOR:
Name=Cu(2+); Xref=ChEBI:CHEBI:29036; Evidence={ECO:0000250};
Name=Zn(2+); Xref=ChEBI:CHEBI:29105; Evidence={ECO:0000250};
Note=Cu(2+) or Zn(2+). {ECO:0000250};
-!- SUBUNIT: Monomer. {ECO:0000250}.
-!- SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250};
Single-pass type II membrane protein {ECO:0000250}; Intermembrane
side {ECO:0000250}.
-!- DOMAIN: The CHCH domain contains a conserved twin Cys-X(9)-Cys
motif which is required for import and stability of MIA40 in
mitochondria. {ECO:0000250}.
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EMBL; CR382124; CAH00284.1; -; Genomic_DNA.
RefSeq; XP_453188.1; XM_453188.1.
SMR; Q6CSA1; -.
STRING; 284590.XP_453188.1; -.
PRIDE; Q6CSA1; -.
EnsemblFungi; CAH00284; CAH00284; KLLA0_D02706g.
GeneID; 2893354; -.
KEGG; kla:KLLA0_D02706g; -.
eggNOG; KOG4149; Eukaryota.
eggNOG; ENOG4111V0I; LUCA.
InParanoid; Q6CSA1; -.
KO; K17782; -.
OrthoDB; EOG092C567C; -.
Proteomes; UP000000598; Chromosome D.
GO; GO:0016021; C:integral component of membrane; IEA:UniProtKB-KW.
GO; GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell.
GO; GO:0015035; F:protein disulfide oxidoreductase activity; IEA:InterPro.
GO; GO:0045041; P:protein import into mitochondrial intermembrane space; IEA:InterPro.
InterPro; IPR010625; CHCH.
InterPro; IPR039289; CHCHD4.
PANTHER; PTHR21622; PTHR21622; 1.
Pfam; PF06747; CHCH; 1.
PROSITE; PS51808; CHCH; 1.
3: Inferred from homology;
Complete proteome; Disulfide bond; Membrane; Mitochondrion;
Mitochondrion inner membrane; Oxidoreductase; Protein transport;
Redox-active center; Reference proteome; Signal-anchor;
Transit peptide; Translocation; Transmembrane; Transmembrane helix;
Transport.
TRANSIT 1 23 Mitochondrion. {ECO:0000255}.
CHAIN 24 406 Mitochondrial intermembrane space import
and assembly protein 40.
/FTId=PRO_0000235291.
TOPO_DOM 24 37 Mitochondrial matrix. {ECO:0000255}.
TRANSMEM 38 54 Helical; Signal-anchor for type II
membrane protein. {ECO:0000255}.
TOPO_DOM 55 406 Mitochondrial intermembrane.
{ECO:0000255}.
DOMAIN 290 334 CHCH. {ECO:0000255|PROSITE-
ProRule:PRU01150}.
MOTIF 293 303 Cx9C motif 1. {ECO:0000255|PROSITE-
ProRule:PRU01150}.
MOTIF 316 326 Cx9C motif 2. {ECO:0000255|PROSITE-
ProRule:PRU01150}.
DISULFID 282 284 Redox-active. {ECO:0000250}.
DISULFID 293 326 {ECO:0000255|PROSITE-ProRule:PRU01150}.
DISULFID 303 316 {ECO:0000255|PROSITE-ProRule:PRU01150}.
SEQUENCE 406 AA; 43212 MW; 5BD597E307CCA047 CRC64;
MFRVSLQATR RVAFRSARQI RFYSAHPPSG GVSHMNKPAL LLAGFSTLGA IYVADGCPTL
IERKPAPAEK PAEETPAAGQ SQSISEPTKD ADESPVSAQE EGAEPVTTPE NEITYSEETH
QALAATLSGD EPAAIEPVAE SVNEQATEPA ASGEATNEPV TGISEDTKAP SLSFDDSKTA
KGVVLEDEAD KKEIQQTSPD AVKTASKDGS EGESDVVLHE KSPAEAETIT EAEEQAEIRS
ISGGTAEQSA TAAAAAAGVQ GEKKNEQQTA YNPETGEINW DCPCLGGMAY GPCGEEFKSA
FSCFVYSEAD PKGINCVEKF STMQNCFRKY PDYYAEQIKD EEEASAEASK IEDKSTTPVS
TATSTVEVQT ENAVFEPVLE KYVEENPQLK DTPEAAAVTN TDDEKK


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