MIA40:ERV1 oxidizes cysteine residues to cystine disulfide bonds

Stable Identifier
R-SCE-1252260
Type
Reaction [transition]
Species
Saccharomyces cerevisiae
Compartment
ReviewStatus
5/5
General
SVG |   | PPTX  | SBGN
MIA40:ERV1 oxidizes cysteine residues to cystine disulfide bonds
MIA40 catalyzes the oxidation of cysteine residues in precursor proteins in the intermembrane space to form cystine bonds (Chacinska et al. 2004, Terziyska et al. 2005, Milenkovic 2007, Sideris and Tokatlidis 2007, Terziyska et al. 2007, Misecke et al. 2008, Muller et al. 2008, Milenkovic et al. 2009, Stojanovski et al. 2008, Longen et al. 2009, Milenkovic et al. 2009, Reddehase et al. 2009, Sideris et al. 2009, Terziyska et al. 2009, Tienson et al. 2009, Bien et al. 2010). The electrons from the cysteine residues are transferred to MIA40, then to ERV1, and eventually to the respiratory chain (Allen et al. 2005, Bihlmaier et al. 2007). The interaction between MIA40 and ERV1 appears to be transitory (Stojanovski et al. 2008). Substrates of MIA40:ERV1 include COX17 (Mesecke et al. 2008, Sideris et al. 2009), COX19 (Bien et al. 2010), CMC2 (Longen et al. 2009), CMC3 (Longen et al. 2009), CMC4 (Longen et al. 2009), TIM13 (Terziyska et al. 2005, Muller et al. 2008, Stojanovski et al. 2008), TIM9 (Chacinska et al. 2004, Milenkovic et al. 2007, Stojanovski et al. 2008, Milenkovic et al. 2009), TIM10 (Chacinska et al. 2004, Milenkovic et al. 2007, Sideris and Tokatlidis 2007, Stojanovski et al. 2008, Terziyska et al. 2009, Milenkovic et al. 2009), CCS1 (Stojanovski et al. 2008, Reddehase et al. 2009), TIM8 (Muller et al. 2008, Stojanovski et al. 2008), and ERV1 (Terziyska et al. 2007).
Literature References
PubMed ID Title Journal Year
17336303 The sulfhydryl oxidase Erv1 is a substrate of the Mia40-dependent protein translocation pathway

Grumbt, B, Bien, M, Neupert, W, Terziyska, N, Hell, K, Herrmann, JM

FEBS Lett 2007
18852299 Mitochondrial protein import: precursor oxidation in a ternary complex with disulfide carrier and sulfhydryl oxidase

Schulze-Specking, A, Pfanner, N, Milenkovic, D, Müller, JM, Baker, MJ, Stojanovski, D, Ryan, MT, Guiard, B, Gabriel, K, Chacinska, A

J Cell Biol 2008
19703468 Systematic analysis of the twin cx(9)c protein family

Westermann, B, Bien, M, Longen, S, Hammermeister, M, Riemer, J, Herrmann, JM, Bihlmaier, K, Kauff, F, Kloeppel, C

J Mol Biol 2009
15359280 Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins

Schulze-Specking, A, Pfanner, N, Kozjak, V, Wiedemann, N, Guiard, B, Sanjuán Szklarz, LK, Pfannschmidt, S, Chacinska, A, Meisinger, C, Truscott, KN

EMBO J 2004
15620710 Mia40, a novel factor for protein import into the intermembrane space of mitochondria is able to bind metal ions

Mesecke, N, Neupert, W, Terziyska, N, Hell, K, Herrmann, JM, Mokranjac, D, Kozany, C, Lutz, T

FEBS Lett 2005
18787558 The zinc-binding protein Hot13 promotes oxidation of the mitochondrial import receptor Mia40

Grumbt, B, Mesecke, N, Longen, S, Terziyska, N, Herrmann, JM, Hell, K, Bihlmaier, K

EMBO Rep 2008
17553782 Biogenesis of the essential Tim9-Tim10 chaperone complex of mitochondria: site-specific recognition of cysteine residues by the intermembrane space receptor Mia40

Schulze-Specking, A, Pfanner, N, Milenkovic, D, Guiard, B, Gabriel, K, Chacinska, A

J Biol Chem 2007
21865594 Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol

Fiedler, S, Petrungaro, C, Longen, S, Suzuki, Y, Riemer, J, Klöppel, C, Kojer, K, Keller, S

Mol Biol Cell 2011
19297525 Identification of the signal directing Tim9 and Tim10 into the intermembrane space of mitochondria

Schulze-Specking, A, Ramming, T, Milenkovic, D, Müller, JM, Stojanovski, D, Rospert, S, Gebert, N, Chacinska, A, Wenz, LS

Mol Biol Cell 2009
19010334 The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1

Grumbt, B, Neupert, W, Reddehase, S, Hell, K

J Mol Biol 2009
20188670 Mitochondrial disulfide bond formation is driven by intersubunit electron transfer in Erv1 and proofread by glutathione

Bien, M, Longen, S, Riemer, J, Herrmann, JM, Wagener, N, Chwalla, I

Mol Cell 2010
16185707 Erv1 mediates the Mia40-dependent protein import pathway and provides a functional link to the respiratory chain by shuttling electrons to cytochrome c

Tokatlidis, K, Balabanidou, V, Lisowsky, T, Sideris, DP, Allen, S

J Mol Biol 2005
21865601 Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system

Burgard, CA, Reddehase, S, Culotta, VC, Hell, K, Gross, DP, Leitch, JM

Mol. Biol. Cell 2011
17978092 Precursor oxidation by Mia40 and Erv1 promotes vectorial transport of proteins into the mitochondrial intermembrane space

Pfanner, N, Milenkovic, D, Müller, JM, Guiard, B, Chacinska, A

Mol Biol Cell 2008
17967948 The disulfide relay system of mitochondria is connected to the respiratory chain

Bien, M, Mesecke, N, Terziyska, N, Herrmann, JM, Hell, K, Bihlmaier, K

J Cell Biol 2007
20026652 A novel intermembrane space-targeting signal docks cysteines onto Mia40 during mitochondrial oxidative folding

Gallo, A, Tokatlidis, K, Banci, L, Petrakis, N, Katrakili, N, Ciofi-Baffoni, S, Sideris, DP, Bertini, I, Mikropoulou, D

J Cell Biol 2009
17680986 Oxidative folding of small Tims is mediated by site-specific docking onto Mia40 in the mitochondrial intermembrane space

Tokatlidis, K, Sideris, DP

Mol Microbiol 2007
17095012 Novel mitochondrial intermembrane space proteins as substrates of the MIA import pathway

Pfanner, N, Milenkovic, D, Müller, J, Guiard, B, Gabriel, K, Chacinska, A, Meisinger, C

J Mol Biol 2007
19011240 Structural and functional roles of the conserved cysteine residues of the redox-regulated import receptor Mia40 in the intermembrane space of mitochondria

Grumbt, B, Terziyska, N, Hell, K, Kozany, C

J Biol Chem 2009
19477928 Reconstitution of the mia40-erv1 oxidative folding pathway for the small tim proteins

Tienson, HL, Kim, SK, Dabir, DV, Hasson, SA, Loo, R, Loo, JA, Koehler, CM, Boontheung, P, Neal, SE

Mol Biol Cell 2009
20367271 The N-terminal shuttle domain of Erv1 determines the affinity for Mia40 and mediates electron transfer to the catalytic Erv1 core in yeast mitochondria

Tokatlidis, K, Pozidis, C, Lionaki, E, Aivaliotis, M

Antioxid Redox Signal 2010
Participants
Catalyst Activity

protein-disulfide reductase activity of MIA40:ERV1 [mitochondrial intermembrane space]

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