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Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells.

Fischer M, Horn S, Belkacemi A, Kojer K, Petrungaro C, Habich M, Ali M, Küttner V, Bien M, Kauff F, Dengjel J, Herrmann JM, Riemer J.

Mol Biol Cell. 2013 Jul;24(14):2160-70. doi: 10.1091/mbc.E12-12-0862.


Structural and functional roles of the conserved cysteine residues of the redox-regulated import receptor Mia40 in the intermembrane space of mitochondria.

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

J Biol Chem. 2009 Jan 16;284(3):1353-63. doi: 10.1074/jbc.M805035200.


Mitochondrial protein import: Mia40 facilitates Tim22 translocation into the inner membrane of mitochondria.

Wrobel L, Trojanowska A, Sztolsztener ME, Chacinska A.

Mol Biol Cell. 2013 Mar;24(5):543-54. doi: 10.1091/mbc.E12-09-0649.


Kinetic control by limiting glutaredoxin amounts enables thiol oxidation in the reducing mitochondrial intermembrane space.

Kojer K, Peleh V, Calabrese G, Herrmann JM, Riemer J.

Mol Biol Cell. 2015 Jan 15;26(2):195-204. doi: 10.1091/mbc.E14-10-1422.


Disulfide bond formation: sulfhydryl oxidase ALR controls mitochondrial biogenesis of human MIA40.

Sztolsztener ME, Brewinska A, Guiard B, Chacinska A.

Traffic. 2013 Mar;14(3):309-20. doi: 10.1111/tra.12030.


Precursor oxidation by Mia40 and Erv1 promotes vectorial transport of proteins into the mitochondrial intermembrane space.

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

Mol Biol Cell. 2008 Jan;19(1):226-36. Erratum in: Mol Biol Cell. 2014 Apr;25(8):1408.


The MIA pathway: a key regulator of mitochondrial oxidative protein folding and biogenesis.

Mordas A, Tokatlidis K.

Acc Chem Res. 2015 Aug 18;48(8):2191-9. doi: 10.1021/acs.accounts.5b00150.


The mitochondrial intermembrane space oxireductase Mia40 funnels the oxidative folding pathway of the cytochrome c oxidase assembly protein Cox19.

Fraga H, Bech-Serra JJ, Canals F, Ortega G, Millet O, Ventura S.

J Biol Chem. 2014 Apr 4;289(14):9852-64. doi: 10.1074/jbc.M114.553479.


The disulfide relay of the intermembrane space oxidizes the ribosomal subunit mrp10 on its transit into the mitochondrial matrix.

Longen S, Woellhaf MW, Petrungaro C, Riemer J, Herrmann JM.

Dev Cell. 2014 Jan 13;28(1):30-42. doi: 10.1016/j.devcel.2013.11.007.


Targeting and maturation of Erv1/ALR in the mitochondrial intermembrane space.

Kallergi E, Andreadaki M, Kritsiligkou P, Katrakili N, Pozidis C, Tokatlidis K, Banci L, Bertini I, Cefaro C, Ciofi-Baffoni S, Gajda K, Peruzzini R.

ACS Chem Biol. 2012 Apr 20;7(4):707-14. doi: 10.1021/cb200485b.


Mitochondrial disulfide relay: redox-regulated protein import into the intermembrane space.

Herrmann JM, Riemer J.

J Biol Chem. 2012 Feb 10;287(7):4426-33. doi: 10.1074/jbc.R111.270678. Review.


The sulfhydryl oxidase Erv1 is a substrate of the Mia40-dependent protein translocation pathway.

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

FEBS Lett. 2007 Mar 20;581(6):1098-102.


Targeting and import mechanism of coiled-coil helix coiled-coil helix domain-containing protein 3 (ChChd3) into the mitochondrial intermembrane space.

Darshi M, Trinh KN, Murphy AN, Taylor SS.

J Biol Chem. 2012 Nov 16;287(47):39480-91. doi: 10.1074/jbc.M112.387696.


Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system.

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

Mol Biol Cell. 2011 Oct;22(20):3758-67. doi: 10.1091/mbc.E11-04-0296.


Biogenesis of yeast Mia40 - uncoupling folding from import and atypical recognition features.

Chatzi A, Sideris DP, Katrakili N, Pozidis C, Tokatlidis K.

FEBS J. 2013 Oct;280(20):4960-9. doi: 10.1111/febs.12482.


Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway.

Neal SE, Dabir DV, Tienson HL, Horn DM, Glaeser K, Ogozalek Loo RR, Barrientos A, Koehler CM.

J Biol Chem. 2015 Aug 21;290(34):20804-14. doi: 10.1074/jbc.M115.669440.


A disulfide relay system in the intermembrane space of mitochondria that mediates protein import.

Mesecke N, Terziyska N, Kozany C, Baumann F, Neupert W, Hell K, Herrmann JM.

Cell. 2005 Jul 1;121(7):1059-69.


Mitochondrial disulfide bond formation is driven by intersubunit electron transfer in Erv1 and proofread by glutathione.

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

Mol Cell. 2010 Feb 26;37(4):516-28. doi: 10.1016/j.molcel.2010.01.017.


Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol.

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

Mol Biol Cell. 2011 Oct;22(20):3749-57. doi: 10.1091/mbc.E11-04-0293.

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