Document Detail

MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria.
MedLine Citation:
PMID:  19182799     Owner:  NLM     Status:  MEDLINE    
MIA40 has a key role in oxidative protein folding in the mitochondrial intermembrane space. We present the solution structure of human MIA40 and its mechanism as a catalyst of oxidative folding. MIA40 has a 66-residue folded domain made of an alpha-helical hairpin core stabilized by two structural disulfides and a rigid N-terminal lid, with a characteristic CPC motif that can donate its disulfide bond to substrates. The CPC active site is solvent-accessible and sits adjacent to a hydrophobic cleft. Its second cysteine (Cys55) is essential in vivo and is crucial for mixed disulfide formation with the substrate. The hydrophobic cleft functions as a substrate binding domain, and mutations of this domain are lethal in vivo and abrogate binding in vitro. MIA40 represents a thioredoxin-unrelated, minimal oxidoreductase, with a facile CPC redox active site that ensures its catalytic function in oxidative folding in mitochondria.
Lucia Banci; Ivano Bertini; Chiara Cefaro; Simone Ciofi-Baffoni; Angelo Gallo; Manuele Martinelli; Dionisia P Sideris; Nitsa Katrakili; Kostas Tokatlidis
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2009-02-01
Journal Detail:
Title:  Nature structural & molecular biology     Volume:  16     ISSN:  1545-9985     ISO Abbreviation:  Nat. Struct. Mol. Biol.     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-02-04     Completed Date:  2009-02-23     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101186374     Medline TA:  Nat Struct Mol Biol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  198-206     Citation Subset:  IM    
Magnetic Resonance Center CERM, University of Florence, Via Luigi Sacconi 6, 50019, Sesto Fiorentino, Florence, Italy.
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MeSH Terms
Amino Acid Sequence
Mitochondria / metabolism*
Mitochondrial Membrane Transport Proteins / chemistry*,  metabolism*
Models, Molecular
Molecular Sequence Data
Nuclear Magnetic Resonance, Biomolecular
Protein Conformation
Protein Folding
Sequence Alignment
Grant Support
Reg. No./Substance:
0/MIA40 protein, human; 0/Mitochondrial Membrane Transport Proteins

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