The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond
Disulfide bond formation drives protein import of most proteins of the mitochondrial intermembrane space (IMS). The main components of this disulfide relay machinery are the oxidoreductase Mia40 and the sulfhydryl oxidase Erv1/ALR. Their precise functions have been elucidated in molecular detail for...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | International Journal of Cell Biology |
| Online Access: | http://dx.doi.org/10.1155/2013/742923 |
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| author | Manuel Fischer Jan Riemer |
| author_facet | Manuel Fischer Jan Riemer |
| author_sort | Manuel Fischer |
| collection | DOAJ |
| description | Disulfide bond formation drives protein import of most proteins of the mitochondrial intermembrane space (IMS). The main components of this disulfide relay machinery are the oxidoreductase Mia40 and the sulfhydryl oxidase Erv1/ALR. Their precise functions have been elucidated in molecular detail for the yeast and human enzymes in vitro and in intact cells. However, we still lack knowledge on how Mia40 and Erv1/ALR impact cellular and organism physiology and whether they have functions beyond their role in disulfide bond formation. Here we summarize the principles of oxidation-dependent protein import mediated by the mitochondrial disulfide relay. We proceed by discussing recently described functions of Mia40 in the hypoxia response and of ALR in influencing mitochondrial morphology and its importance for tissue development and embryogenesis. We also include a discussion of the still mysterious function of Erv1/ALR in liver regeneration. |
| format | Article |
| id | doaj-art-744bafa950b14925bff85034d40ddeff |
| institution | Kabale University |
| issn | 1687-8876 1687-8884 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Cell Biology |
| spelling | doaj-art-744bafa950b14925bff85034d40ddeff2025-02-03T05:58:35ZengWileyInternational Journal of Cell Biology1687-88761687-88842013-01-01201310.1155/2013/742923742923The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and BeyondManuel Fischer0Jan Riemer1Cellular Biochemistry, University of Kaiserslautern, Erwin-Schrödinger Straße 13, 67663 Kaiserslautern, GermanyCellular Biochemistry, University of Kaiserslautern, Erwin-Schrödinger Straße 13, 67663 Kaiserslautern, GermanyDisulfide bond formation drives protein import of most proteins of the mitochondrial intermembrane space (IMS). The main components of this disulfide relay machinery are the oxidoreductase Mia40 and the sulfhydryl oxidase Erv1/ALR. Their precise functions have been elucidated in molecular detail for the yeast and human enzymes in vitro and in intact cells. However, we still lack knowledge on how Mia40 and Erv1/ALR impact cellular and organism physiology and whether they have functions beyond their role in disulfide bond formation. Here we summarize the principles of oxidation-dependent protein import mediated by the mitochondrial disulfide relay. We proceed by discussing recently described functions of Mia40 in the hypoxia response and of ALR in influencing mitochondrial morphology and its importance for tissue development and embryogenesis. We also include a discussion of the still mysterious function of Erv1/ALR in liver regeneration.http://dx.doi.org/10.1155/2013/742923 |
| spellingShingle | Manuel Fischer Jan Riemer The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond International Journal of Cell Biology |
| title | The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond |
| title_full | The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond |
| title_fullStr | The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond |
| title_full_unstemmed | The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond |
| title_short | The Mitochondrial Disulfide Relay System: Roles in Oxidative Protein Folding and Beyond |
| title_sort | mitochondrial disulfide relay system roles in oxidative protein folding and beyond |
| url | http://dx.doi.org/10.1155/2013/742923 |
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