MIA40_YEAST » Mitochondrial intermembrane space import and assembly protein 40

MIA40_YEAST » Mitochondrial intermembrane space import and assembly protein 40
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Topology in Mitochondrial inner membrane
Topologyintermembrane space
mitochondrial matrix side
MIA40_YEAST » Mitochondrial intermembrane space import and assembly protein 40 » Mitochondrial import inner membrane translocase TIM40;
Hydrophobic Thickness 27.2 ± 2.7 Å
Tilt Angle 13 ± 3°
ΔGtransfer -15.1 kcal/mol
ΔGfold -7.4 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING
Topology In
TM Segments 46-64 (43-65)
Pathways

Mitochondrial Protein Import (yeast) (Reactome)

PDB 3a3c (282-353), 2zxt (282-365)
OPM none
Complexes none
Interactions

COX7, Complex: COX7:MIA40

CY1, Complex: CY1:MIA40

DPM1, Complex: DPM1:MIA40, PubMed

FCJ1, Complex: MIA40:FCJ1, PubMed

KRE2, Complex: KRE2:MIA40, PubMed

OST1, Complex: OST1:MIA40, PubMed

QCR9, Complex: QCR9:MIA40

TIM14, Complex: TIM14:MIA40

TIM21, Complex: DYR:CYB2:MIA40:TIM21:TIM17, PubMed

TOM22, Complex: TOM22:MIA40, PubMed

TOM70, Complex: TOM70:MIA40

Domains

AA: 307-343, PDBID: 2ZXT, Subunit A, Seq Identity:100%, CHCH domain

UniProt annotation for MIA40_YEAST » Mitochondrial intermembrane space import and assembly protein 40
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. Reduced MIA40 is reoxidized by FAD-linked sulfhydryl oxidase ERV1.

SUBUNIT: Monomer. Interacts with the FAD-linked sulfhydryl oxidase ERV1 and with the substrate proteins COX17, TIM9, and TIM13, forming transient intermolecular disulfide bridges. Interacts with FCJ1.

DOMAIN: The CHCH domain contains a conserved twin Cys-X(9)-Cys motif which is required for import and stability of MIA40 in mitochondria.

MISCELLANEOUS: Present with 5040 molecules/cell in log phase SD medium.

UniProt features for MIA40_YEAST » Mitochondrial intermembrane space import and assembly protein 40
TRANSIT 1 31 Mitochondrion.
CHAIN 32 403 Mitochondrial intermembrane space import and assembly protein 40.
DOMAIN 300 343 CHCH.
DISULFID 296 298 Redox-active.
DISULFID 307 340
DISULFID 317 330
Amino Acid Sequence for MIA40_YEAST » Mitochondrial intermembrane space import and assembly protein 40
MLRNLVVRNA CRNRPSIQVA RGLCRHQTRR LMASSPQFGR NSNQEKTAGF IMGILSMAGA LYFIAPNRKP LFASRKVESD KTAEEELSSG GEQSPENEDD NNSKSDENGD DNDSKNDETE AGPQLGGDKI GASKVAEDGE LVVLAEEDNK SSEDKDTDES KVSTKDDEQS NEDNATANNQ KDENISSENS EENTSDKTLD NNAGSSEKKD PEHSDDEKSQ QGQSDDKTTT EDNNGEEESS KKTVSDSENS AKQSESSDEE KEELRKQEEK QMGPTEEEVQ HEGAYNPDTG EINWDCPCLG GMAHGPCGEE FKSAFSCFVY SEAEPKGIDC VEKFQHMQDC FRKYPEHYAE QLKETSDDEE PQDKVKVNTI ESAPNVSSAK ENAAKKAEQS DVKKEPLNEE SKP