Research group of Nils Wiedemann

Prof. Dr. Nils Wiedemann

Institute for Biochemistry and Molecular Biology
Stefan-Meier-Straße 17
79104 Freiburg, Germany
+49 (0) 761 203 5280
nils.wiedemann(at)biochemie.uni-freiburg.de

Research

Mitochondria are endosymbiotic organelles essential for the oxidative energy metabolism of eukaryotic cells. Similar to Gram-negative bacteria, mitochondria contain an outer and an inner membrane and an own genome. However, only a handful of proteins are encoded in mitochondria. In addition, no lipids are synthesized de novo within mitochondria. Therefore the vast majority of mitochondrial proteins and lipid precursors must be imported and we study the biogenesis of mitochondrial membranes.

Schema Membranbiogenese Mitochondrien — Mechanisms required for mitochondrial membrane biogenesis

Video about our research

Projects

Import and insertion mitochondrial membrane proteins
Assembly of respiratory chain complexes
Connection between mitochondria and the endoplasmic reticulum
Biogenesis of mitochondrial lipids
Analysis of mutants affecting the mitochondrial energy metabolism
Organization of mitochondrial DNA-protein complexes
Identification and analysis of novel mitochondrial proteins encoded by small open reading frames

MITOsmORFs: Identification and analysis of novel mitochondrial proteins encoded by small open reading frames

Compared to other cellular compartments, mitochondria exhibit a remarkably high content of small proteins. Approximately one-third of the cellular proteins characterized to date with a size of ≤15 kDa are located in mitochondria.

These small proteins play a role in all aspects of mitochondrial biology, e.g., they influence cristae morphology, the formation of Fe-S clusters, the transport of metabolic molecules, protein production, and cellular respiration.

However, the precise intracellular localization and function of most small proteins is unknown. In the model organism budding yeast, these small proteins account for one-third of the uncharacterized open reading frames. Collectively, we estimate that more than 10% of the mitochondrial proteome remains to be discovered.

In an initial study, we demonstrated the mitochondrial localization of several uncharacterized small open reading frame (smORF) proteins.

Due to their critical role in cellular energy metabolism, mitochondria are considered the powerhouses of the cell. Mitochondria convert energy from food and generate more than 90% of cellular adenosine triphosphate (ATP), the main driving force of cellular metabolism. In humans, dysfunction of mitochondrial proteins causes severe encephalomyopathies, often associated with multiorgan dysfunction and neurodegenerative diseases.

To understand mitochondrial functions, it is essential to determine the composition of mitochondrial proteins. MITOsmORFs aims to identify over 100 novel mitochondrial proteins. Furthermore, MITOsmORFs will determine the sub-mitochondrial localization and functional role of mitochondrial proteins. This includes mapping interactions and characterizing mitochondrial activities in vivo and in organelles to explore the unknown biology of mitochondrial proteins.

Team

Name	Phone +49 (0) 761 203	Email @biochemie.uni-freiburg.de
Nils Wiedemann Dr.	-5280	nils.wiedemann
Jakob Busch, Dr.	-5243	jakob.busch
Iniyan Ganesan, PhD	-5237	iniyan.ganesan
Laura Melchionda, PhD	-5227	laura.melchionda
Conny Steiert	-5238	conny.steiert
Nicole Zufall	-5239	nicole.zufall

CV Nils Wiedemann

Since 2015	Apl. Professor of Biochemistry and Molecular Biology, Faculty of Medicine, University of Freiburg
2010	Habilitation in Biochemistry and Molecular Biology, Faculty of Medicine, University of Freiburg
2006	Visiting Scientist, La Trobe University, Melbourne, Australia
2004 ‑ 2015	Group Leader, Institute of Biochemistry and Molecular Biology, University of Freiburg
2002 ‑ 2004	Postdoc, Institute of Biochemistry and Molecular Biology, University of Freiburg
2002	Dr. rer. nat. (summa cum laude), Department of Biology, University of Freiburg
2001	Yeast Genetics Course, Cold Spring Harbor Laboratory, USA
1999	Diploma in Chemistry, Department of Chemistry, University of Freiburg
1995 ‑ 1999	Studied Chemistry, Albert Ludwig University of Freiburg
1995 ‑ 1999	Studied Biochemistry, Cell Biology, and Molecular Biology (Master’s programs), University of Massachusetts Amherst, USA
1993 ‑ 1995	Studied Chemistry, Johann Wolfgang Goethe University Frankfurt

Honors

2015	ERC Consolidator Grant
2011	Eugen-Graetz Research Award, University of Freiburg
2007	Young Investigator Award, German Society for Biochemistry and Molecular Biology (GBM), Frankfurt
2003	Hans Grisebach Award for Dissertation, Faculty of Biology, University of Freiburg
2002	summa cum laude, University of Freiburg

Funding

The Wiedemann working group receives research funding from the following funding programs of the German Research Foundation (DFG)

Collaborative Research Centre (CRC) 1381: Protein Machineries since 2019, extended 2023
Cluster of Excellence (EXC) CIBSS – Centre for Integrative Biological Signalling Studies since 2019
EXC BIOSS Centre for Biological Signalling Studies 2012-1018
CRC 1140: Kidney Disease – From Genes to Mechanisms KIDGEM 2015-2018

Through the European Union

European Research Council (ERC) Consolidator grant “MITOsmORFs: Identification and analysis of novel mitochondrial proteins encoded by small open reading frames” for Nils Wiedemann 2015-2021

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Ganesan I, Busto JV, Pfanner N, Wiedemann N (2024). Biogenesis of mitochondrial β-barrel membrane proteins. FEBS Open Bio. doi: 10.1002/2211-5463.13905

Busto JV, Ganesan I, Mathar H, Steiert C, Schneider EF, Straub SP, Ellenrieder L, Song J, Stiller SB, Lübbert P, Chatterjee R, Elsaesser J, Melchionda L, Schug C, den Brave F, Schulte U, Klecker T, Kraft C, Fakler B, Becker T, Wiedemann N (2024). Role of the small protein Mco6 in the mitochondrial sorting and assembly machinery. Cell Rep. doi: 10.1016/j.celrep.2024.113805

Ganesan I, Pfanner N, Wiedemann N (2024).
Screen for temperature-sensitive mutants of non-essential yeast genes. Methods Enzymol. doi: 10.1016/bs.mie.2024.07.040

Fielden LF, Busch JD, Lindau C, Qiu J, Wiedemann N (2024). Analysis of mitochondrial protein translocation by disulfide bond formation and cysteine specific crosslinking. Methods Enzymol. doi: 10.1016/bs.mie.2024.07.057

Steiert C, Busto JV, Melchionda L, Wiedemann N (2024). Subcellular fractionation by differential centrifugation for mitochondrial studies. Methods Enzymol. doi: 10.1016/bs.mie.2024.07.037

Fielden LF, Busch JD, Merkt SG, Ganesan I, Steiert C, Hasselblatt HB, Busto JV, Wirth C, Zufall N, Jungbluth S, Noll K, Dung JM, Butenko L, von der Malsburg K, Koch HG, Hunte C, van der Laan M, Wiedemann N (2023). Central role of Tim17 in mitochondrial presequence protein translocation. Nature. doi: 10.1038/s41586-023-06477-8

Busch JD, Fielden LF, Pfanner N, Wiedemann N (2023). Mitochondrial protein transport: Versatility of translocases and mechanisms. Mol Cell. doi: 10.1016/j.molcel.2023.02.020

Schulte U, den Brave F, Haupt A, Gupta A, Song J, Müller CS, Engelke J, Mishra S, Mårtensson C, Ellenrieder L, Priesnitz C, Straub SP, Doan KN, Kulawiak B, Bildl W, Rampelt H, Wiedemann N, Pfanner N, Fakler B, Becker T (2023). Mitochondrial complexome reveals quality-control pathways of protein import. Nature. doi: 10.1038/s41586-022-05641-w

Takeda H, Busto JV, Lindau C, Tsutsumi A, Tomii K, Imai K, Yamamori Y, Hirokawa T, Motono C, Ganesan I, Wenz LS, Becker T, Kikkawa M, Pfanner N, Wiedemann N, Endo T (2023). A multipoint guidance mechanism for β-barrel folding on the SAM complex. Nat Struct Mol Biol. doi: 10.1038/s41594-022-00897-2

Morgenstern M, Peikert CD, Lübbert P, Suppanz I, Klemm C, Alka O, Steiert C, Naumenko N, Schendzielorz A, Melchionda L, Mühlhäuser WWD, Knapp B, Busch JD, Stiller SB, Dannenmaier S, Lindau C, Licheva M, Eickhorst C, Galbusera R, Zerbes RM, Ryan MT, Kraft C, Kozjak-Pavlovic V, Drepper F, Sven Dennerlein S, Oeljeklaus S, Pfanner N, Wiedemann N*, Warscheid B*: Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context. Cell Metabolism, 2021;33(12): 2464-2483.e18. * Corresponding
Takeda H, Tsutsumi A, Nishizawa T, Lindau C, Busto JV, Wenz LS, Ellenrieder L, Imai K, Straub SP, Mossmann W, Qiu J, Yamamori Y, Tomii K, Suzuki J, Murata T, Ogasawara S, Nureki O, Becker T, Pfanner N, Wiedemann N, Kikkawa M, Endo T: Mitochondrial sorting and assembly machinery operates by β-barrel switching. Nature, 2021;590(7844):163-169.
Ghosh SG, Lee S, Fabunan R, Chai G, Zaki MS, Abdel-Salam G, Sultan T, Ben-Omran T, Alvi JR, McEvoy-Venneri J, Stanley V, Patel A, Ross D, Ding J, Jain M, Pan D, Lübbert P, Kammerer B, Wiedemann N, Verhoeven-Duif NM, Jans JJ, Murphy D, Toosi MB, Ashrafzadeh F, Imannezhad S, Karimiani EG, Ibrahim K, Waters ER, Maroofian R, Gleeson JG: Biallelic variants in HPDL, encoding 4-hydroxyphenylpyruvate dioxygenase-like protein, lead to an infantile neurodegenerative condition. Genet Med, 2021;23(3):524-533.

Rampelt H, Sucec I, Bersch B, Horten P, Perschil I, Martinou JC, van der Laan M, Wiedemann N, Schanda P, Pfanner N: The mitochondrial carrier pathway transports non-canonical substrates with an odd number of transmembrane segments. BMC Biol, 2020;18(1):2.

Araiso Y, Tsutsumi A, Qiu J, Imai K, Shiota T, Song J, Lindau C, Wenz LS, Sakaue H, Yunoki K, Kawano S, Suzuki J, Wischnewski M, Schütze C, Ariyama H, Ando T, Becker T, Lithgow T, Wiedemann N, Pfanner N, Kikkawa M, Endo T: Structure of the mitochondrial import gate reveals distinct preprotein paths. Nature, 2019;575(7782):395-401.
Pan D, Wiedemann N*, Kammerer B*: Heat Stress-Induced Metabolic Remodeling in Saccharomyces cerevisiae. Metabolites, 2019;9(11):266. *Corresponding
Pfanner N, Warscheid B, Wiedemann N: Mitochondrial proteins: from biogenesis to functional networks. Nat Rev Mol Cell Biol, 2019;20(5):267-284.

Weinhäupl K, Lindau C, Hessel A, Wang Y, Schütze C, Jores T, Melchionda L, Schönfisch B, Kalbacher H, Bersch B, Rapaport D, Brennich M, Lindorff-Larsen K, Wiedemann N*, Schanda P*: Structural Basis of Membrane Protein Chaperoning through the Mitochondrial Intermembrane Space. Cell, 2018;175(5):1365-1379.e25. *Corresponding
Dannenmaier S, Stiller SB, Morgenstern M, Lübbert P, Oeljeklaus S, Wiedemann N*, Warscheid B*: Complete Native Stable Isotope Labeling by Amino Acids of Saccharomyces cerevisiae for Global Proteomic Analysis. Anal Chem, 2018;90(17):10501-10509. *Corresponding
Hofherr A, Seger C, Fitzpatrick F, Busch T, Michel E, Luan J, Osterried L, Linden F, Kramer-Zucker A, Wakimoto B, Schütze C, Wiedemann N, Artati A, Adamski J, Walz G, Kunji ERS, Montell C, Watnick T, Köttgen M: The mitochondrial transporter SLC25A25 links ciliary TRPP2 signaling and cellular metabolism. PLoS Biol, 2018;16(8):e2005651.
Böttinger L, Mårtensson CU, Song J, Zufall N, Wiedemann N, Becker T: Respiratory chain supercomplexes associate with the cysteine desulfurase complex of the iron-sulfur cluster assembly machinery. Mol Biol Cell, 2018;29(7):776-785.
Pan D, Lindau C, Lagies S, Wiedemann N*, Kammerer B*: Metabolic profiling of isolated mitochondria and cytoplasm reveals compartment-specific metabolic responses. Metabolomics, 2018;14(5):59. * Corresponding
Höhr AIC, Lindau C, Wirth C, Qiu J, Stroud DA, Kutik S, Guiard B, Hunte C, Becker T, Pfanner N, Wiedemann N: Membrane protein insertion through a mitochondrial β-barrel gate. Science, 2018;359(6373):eaah6834.
Abstract Full text PDF

Krüger V, Becker T, Becker L, Montilla-Martinez M, Ellenrieder L, Vögtle FN, Meyer HE, Ryan MT, Wiedemann N, Warscheid B, Pfanner N, Wagner R, Meisinger C: Identification of new channels by systematic analysis of the mitochondrial outer membrane. J Cell Biol,2017; 216(11):3485-3495.
Morgenstern M, Stiller, SB, Lübbert P, Peikert, CD, Dannenmaier, S, Drepper F, Weill U, Höß P, Feuerstein R, Gebert M, Bohnert M, van der Laan M, Schuldiner M, Schütze C, Oeljeklaus S, Pfanner N, Wiedemann N*, Warscheid B*: Definition of a High-Confidence Mitochondrial Proteome at Quantitative Scale. Cell Reports, 2017;19(13):2836-2852. *Corresponding
Wiedemann N, and Pfanner N: Mitochondrial Machineries for Protein Import and Assembly. Annu Rev Biochem, 2017;86:685-714.

2016

Stiller SB, Höpker J, Oeljeklaus S, Schütze C, Schrempp SG, Vent-Schmidt J, Horvath, SE, Frazier AE, Gebert N, van der Laan M, Bohnert M, Warscheid B, Pfanner N, and Wiedemann N: Mitochondrial OXA translocase plays a major role in biogenesis of inner-membrane proteins. Cell Metab, 2016;23(5):901-908.
Ellenrieder L, Opalinski L, Becker L, Krüger V, Mirus O, Straub SP, Ebell K, Flinner N, Stiller SB, Guiard B, Meisinger C, Wiedemann N, Schleiff E, Wagner R, Pfanner N, Becker T: Separating mitochondrial protein assembly and endoplasmic reticulum tethering by selective coupling of Mdm10. Nat Commun, 2016;7:13021.
Straub SP, Stiller SB, Wiedemann N, Pfanner N: Dynamic organization of the mitochondrial protein import machinery. Biol Chem, 2016; 397(11):1097-1114.
Yofe I, Weill U, Meurer M, Chuartzman S, Zalckvar E, Goldman O, Ben-Dor S, Schütze C, Wiedemann N, Knop M, Khmelinskii A, Schuldiner M: One library to make them all: streamlining the creation of yeast libraries via a SWAp-Tag strategy. Nat Methods, 2016;13(4):371-378.

2015

Wenz L-S, Ellenrieder L, Qiu J, Bohnert M, Zufall N, van der Laan M, Pfanner N*, Wiedemann N*, Becker T: Sam37 is crucial for formation of the mitochondrial TOM-SAM supercomplex, thereby promoting β-barrel biogenesis. J Cell Biol, 2015;210(7):1047-1054. *Corresponding
Shiota T, Imai K, Qiu J, Hewitt VL, Tan K, Shen H-H, Sakiyama N, Fukasawa Y, Hayat S, Kamiya M, Elofsson A, Tomii K, Horton P, Wiedemann N, Pfanner N, Lithgow T, Endo T: Molecular architecture of the active mitochondrial protein gate. Science, 2015;349(6255):1544-1548.
Vögtle FN, Keller M, Taskin AA, Horvath SE, Guan XL, Prinz C, Opalińska M, Zorzin C, van der Laan M, Wenk MR, Schubert R, Wiedemann N, Holzer M, Meisinger C: The fusogenic lipid phosphatidic acid promotes the biogenesis of mitochondrial outer membrane protein Ugo1. J Cell Biol, 2015;210(6):951-960.
Elbaz-Alon Y, Eisenberg-Bord M, Shinder V, Stiller SB, Shimoni E, Wiedemann N, Geiger T, Schuldiner M: Lam6 Regulates the Extent of Contacts between Organelles. Cell Rep, 2015;12(1):7-14.
Bragoszewski P, Wasilewski M, Sakowska P, Gornicka A, Böttinger L, Qiu J, Wiedemann N, Chacinska A: Retro-translocation of mitochondrial intermembrane space proteins. Proc Natl Acad Sci USA, 2015;112(25):7713-7718.
Wenz L-S, Opaliński Ł, Wiedemann N, Becker T: Cooperation of protein machineries in mitochondrial protein sorting. Biochim Biophys Acta, 2015;1853 (5):1119-1129.
Höhr AI, Straub SP, Warscheid B, Becker T, Wiedemann N: Assembly of β-barrel proteins in the mitochondrial outer membrane. Biochim Biophys Acta, 2015; 1853(1):74-88.

2014

Mossmann D, Vögtle FN, Taskin AA, Teixeira PF, Ring J, Burkhart JM, Burger N, Pinho CM, Tadic J, Loreth D, Graff C, Metzger F, Sickmann A, Kretz O, Wiedemann N, Zahedi RP, Madeo F, Glaser E, Meisinger C: Amyloid-beta Peptide Induces Mitochondrial Dysfunction by Inhibition of Preprotein Maturation. Cell Metabolism, 2014;20(4):1-8.
Wenz L.-S, Opalinski L, Schuler M-H, Ellenrieder L, Ieva, R, Böttinger, L, Qiu J, van der Laan, M, Wiedemann N, Guiard B, Pfanner N, Becker T. The presequence pathway is involved in protein sorting to the mitochondrial outer membrane. EMBO Rep, 2014;15(6):678-85.
Wenz L-S, Qiu J, Becker T, Wiedemann N: Biogenesis and folding of β-barrel membrane proteins. Cell Cycle, 2014;13(2):169-170.

2013

Böttinger L, Guiard B, Oeljeklaus S, Kulawiak B, Zufall N, Wiedemann N, Warscheid B, van der Laan M, Becker T: A complex of Cox4 and mitochondrial Hsp70 plays an important role in the assembly of the cytochrome c oxidase. Mol Biol Cell, 2013;24(17):2609-2619.
Kulawiak B, Hopker J, Gebert M, Guiard B, Wiedemann N*, Gebert N: The mitochondrial protein import machinery has multiple connections to the respiratory chain Biochim Biophys Acta, 2013;1827(5):612-626. *Corresponding
Qiu J, Wenz LS, Zerbes RM, Oeljeklaus S, Bohnert M, Stroud DA, Wirth C, Ellenrieder L, Thornton N, Kutik S, Wiese S, Schulze-Specking A, Zufall N, Chacinska A, Guiard B, Hunte C, Warscheid B, van der Laan M, Pfanner N*, Wiedemann N*, Becker T: Coupling of mitochondrial import and export translocases by receptor-mediated supercomplex formation. Cell, 2013;154(3):596-608. *Corresponding
Wiedemann N, Stiller SB, Pfanner N: Activation and degradation of mitofusins: two pathways regulate mitochondrial fusion by reversible ubiquitylation. Mol Cell, 2013;49(3):423-425.

2012

Bohnert M, Wenz LS, Zerbes RM, Horvath SE, Stroud DA, von der Malsburg K, Muller JM, Oeljeklaus S, Perschil I, Warscheid B, Chacinska A, Veenhuis M, van der Klei IJ, Daum G, Wiedemann N, Becker T, Pfanner N, van der Laan M: Role of mitochondrial inner membrane organizing system in protein biogenesis of the mitochondrial outer membrane. Mol Biol Cell, 2012;23(20):3948-3956.
Horvath SE, Böttinger L, Vögtle FN, Wiedemann N, Meisinger C, Becker T, Daum G: Processing and topology of the yeast mitochondrial phosphatidylserine decarboxylase 1. J Biol Chem, 2012;287(44):36744-36755.
Reinhold R, Krüger V, Meinecke M, Schulz C, Schmidt B, Grunau SD, Guiard B, Wiedemann N, van der Laan M, Wagner R, Rehling P, Dudek J: The channel-forming Sym1 protein is transported by the TIM23 complex in a presequence-independent manner. Mol Cell Biol, 2012;32(24):5009-5021.
Van der Laan M, Bohnert M, Wiedemann N, Pfanner N: Role of MINOS in mitochondrial membrane architecture and biogenesis. Trends Cell Biol, 2012;22(4):185-192.
Zerbes RM, Bohnert M, Stroud DA, von der Malsburg K, Kram A, Oeljeklaus S, Warscheid B, Becker T, Wiedemann N, Veenhuis M, van der Klei IJ, Pfanner N, van der Laan M: Role of MINOS in mitochondrial membrane architecture: cristae morphology and outer membrane interactions differentially depend on mitofilin domains. J Mol Biol, 2012;422(2):183-191.

2011

Becker T, Wenz LS, Thornton N, Stroud D, Meisinger C, Wiedemann N, Pfanner N: Biogenesis of mitochondria: dual role of Tom7 in modulating assembly of the preprotein translocase of the outer membrane. J Mol Biol, 2011;405(1):113-124.
Gebert N, Gebert M, Oeljeklaus S, von der Malsburg K, Stroud DA, Kulawiak B, Wirth C, Zahedi RP, Dolezal P, Wiese S, Simon O, Schulze-Specking A, Truscott KN, Sickmann A, Rehling P, Guiard B, Hunte C, Warscheid B, van der Laan M, Pfanner N, Wiedemann N: Dual function of Sdh3 in the respiratory chain and TIM22 protein translocase of the mitochondrial inner membrane. Mol Cell, 2011;44(5):811-818.
Qian X, Gebert M, Hopker J, Yan M, Li J, Wiedemann N, van der Laan M, Pfanner N, Sha B: Structural basis for the function of Tim50 in the mitochondrial presequence translocase. J Mol Biol, 2011;411(3):513-519.
Stroud DA, Becker T, Qiu J, Stojanovski D, Pfannschmidt S, Wirth C, Hunte C, Guiard B, Meisinger C, Pfanner N, Wiedemann N: Biogenesis of mitochondrial beta-barrel proteins: the POTRA domain is involved in precursor release from the SAM complex. Mol Biol Cell, 2011;22(16):2823-2833.
Stroud DA, Oeljeklaus S, Wiese S, Bohnert M, Lewandrowski U, Sickmann A, Guiard B, van der Laan M, Warscheid B, Wiedemann N: Composition and topology of the endoplasmic reticulum-mitochondria encounter structure. J Mol Biol, 2011;413(4):743-750.
Gebert N, Ryan MT, Pfanner N, Wiedemann N, Stojanovski D: Mitochondrial protein import machineries and lipids: a functional connection Biochim Biophys Acta, 2011;1808(3):1002-1011.

2010

Becker T, Guiard B, Thornton N, Zufall N, Stroud DA, Wiedemann N, Pfanner N: Assembly of the mitochondrial protein import channel: role of Tom5 in two-stage interaction of Tom40 with the SAM complex. Mol Biol Cell, 2010;21(18):3106-3113.
Chacinska A, van der Laan M, Mehnert CS, Guiard B, Mick DU, Hutu DP, Truscott KN, Wiedemann N, Meisinger C, Pfanner N, Rehling P: Distinct forms of mitochondrial TOM-TIM supercomplexes define signal-dependent states of preprotein sorting. Mol Cell Biol, 2010;30(1):307-318.
Stroud DA, Meisinger C, Pfanner N, Wiedemann N: Biochemistry. Assembling the outer membrane. Science, 2010;328(5980):831-832.

2009

Gebert N, Joshi AS, Kutik S, Becker T, McKenzie M, Guan XL, Mooga VP, Stroud DA, Kulkarni G, Wenk MR, Rehling P, Meisinger C, Ryan MT, Wiedemann N, Greenberg ML, Pfanner N: Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome. Curr Biol, 2009;19(24):2133-2139.
Wiedemann N, Meisinger C, Pfanner N: Cell biology. Connecting organelles. Science, 2009;325(5939).403-404.
Kutik S, Stroud DA, Wiedemann N, Pfanner N: Evolution of mitochondrial protein biogenesis. Biochim Biophys Acta, 2009;1790(6):409-415.
Wiedemann N, van der Laan M, Pfanner N: SnapShot: import and sorting of mitochondrial proteins. Cell, 2009;138(4):808-808.e1.

2008

Becker T, Pfannschmidt S, Guiard B, Stojanovski D, Milenkovic D, Kutik S, Pfanner N, Meisinger C, Wiedemann N: Biogenesis of the mitochondrial TOM complex: Mim1 promotes insertion and assembly of signal-anchored receptors. J Biol Chem, 2008;283(1):120-127.
Gebert N, Chacinska A, Wagner K, Guiard B, Koehler CM, Rehling P, Pfanner N, Wiedemann N: Assembly of the three small Tim proteins precedes docking to the mitochondrial carrier translocase. EMBO Rep, 2008;9(6):548-554.
Kutik S, Rissler M, Guan XL, Guiard B, Shui G, Gebert N, Heacock PN, Rehling P, Dowhan W, Wenk MR, Pfanner N, Wiedemann N: The translocator maintenance protein Tam41 is required for mitochondrial cardiolipin biosynthesis. J Cell Biol, 2008;183(7):1213-1221.
Kutik S, Stojanovski D, Becker L, Becker T, Meinecke M, Krüger V, Prinz C, Meisinger C, Guiard B, Wagner R, Pfanner N, Wiedemann N: Dissecting membrane insertion of mitochondrial beta-barrel proteins. Cell, 2008;132(6):1011-1024.
Wagner K, Gebert N, Guiard B, Brandner K, Truscott KN, Wiedemann N, Pfanner N, Rehling P: The assembly pathway of the mitochondrial carrier translocase involves four preprotein translocases. Mol Cell Biol, 2008;28(13):4251-4260.
Kutik S, Stojanovski D, Becker T, Stroud DA, Becker L, Meinecke M, Krüger V, Prinz C, Guiard B, Wagner R, Meisinger C, Pfanner N, Wiedemann N: Response: The Mitochondrial beta-Signal and Protein Sorting. Cell, 2008;135(7):1159-1160.

2007

Kutik S, Guiard B, Meyer HE, Wiedemann N, Pfanner N: Cooperation of translocase complexes in mitochondrial protein import. J Cell Biol, 2007;179(4):585-591.
Meisinger C, Pfannschmidt S, Rissler M, Milenkovic D, Becker T, Stojanovski D, Youngman MJ, Jensen RE, Chacinska A, Guiard B, Pfanner N, Wiedemann N: The morphology proteins Mdm12/Mmm1 function in the major beta-barrel assembly pathway of mitochondria. EMBO J, 2007;26(9):2229-2239.
Wiedemann N, van der Laan M, Hutu DP, Rehling P, Pfanner N: Sorting switch of mitochondrial presequence translocase involves coupling of motor module to respiratory chain. J Cell Biol, 2007;179:1115-1122.
Stojanovski D, Pfannner N, Wiedemann N: Import of proteins into mitochondria. Method Cell Biol, 2007;80:783-806.

2006

Meisinger C, Wiedemann N, Rissler M, Strub A, Milenkovic D, Schönfisch B, Muller H, Kozjak V, Pfanner N: Mitochondrial protein sorting: differentiation of beta-barrel assembly by J Biol Chem, 2006;281(32):22819-22826.
Wiedemann N, Pfanner N, Chacinska A: Chaperoning through the Mitochondrial Intermembrane Space. Mol Cell, 2006; 21(2):145-148.
Wiedemann N, Pfanner N, Rehling P: Import of precursor proteins into isolated yeast mitochondria. Methods Mol Biol, 2006;313:373-384.
Wiedemann N, Urzica E, Guiard B, Muller H, Lohaus C, Meyer HE, Ryan MT, Meisinger C, Muhlenhoff U, Lill R, Pfanner N: Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins. EMBO J, 2006;25(1):184-195.
van der Laan M, Wiedemann N, Mick DU, Guiard B, Rehling P, Pfanner N: A role for Tim21 in membrane-potential-dependent preprotein sorting in mitochondria. Curr Biol, 2006;16(22):2271-2276.

2005

Rissler M, Wiedemann N, Pfannschmidt S, Gabriel K, Guiard B, Pfanner N, Chacinska A: The Essential Mitochondrial Protein Erv1 Cooperates with Mia40 in Biogenesis of Intermembrane Space Proteins. J Mol Biol, 2005;353:485-492.
Sanjuan Szklarz LK, Guiard B, Rissler M, Wiedemann N, Kozjak V, van der Laan M, Lohaus C, Marcus K, Meyer HE, Chacinska A, Pfanner N, Meisinger C: Inactivation of the mitochondrial heat shock protein zim17 leads to aggregation of matrix hsp70s followed by pleiotropic effects on morphology and protein biogenesis. J Mol Biol, 2005;351(1):206-218.

2004

Chacinska A, Pfannschmidt S, Wiedemann N, Kozjak V, Sanjuan Szklarz LK, Schulze-Specking A, Truscott KN, Guiard B, Meisinger C, Pfanner N: Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins. Embo J, 2004;23(19):3735-3746.
Meisinger C, Rissler M, Chacinska A, Szklarz LK, Milenkovic D, Kozjak V, Schönfisch B, Lohaus C, Meyer HE, Yaffe MP, Guiard B, Wiedemann N, Pfanner N: The mitochondrial morphology protein Mdm10 functions in assembly of the preprotein translocase of the outer membrane. Dev Cell, 2004;7(1):61-71.
Milenkovic D, Kozjak V, Wiedemann N, Lohaus C, Meyer HE, Guiard B, Pfanner N, Meisinger C: Sam35 of the mitochondrial protein sorting and assembly machinery is a peripheral outer membrane protein essential for cell viability. J Biol Chem, 2004; 279(21):22781-22785.
Pfanner N, Wiedemann N, Meisinger C: Cell biology. Double membrane fusion. Science, 2004;305(5691):1723-1724.
Pfanner N, Wiedemann N, Meisinger C, Lithgow T: Assembling the mitochondrial outer membrane. Nat Struct Mol Biol, 2004;11(11):1044-1048.
Wiedemann N, Frazier AE, Pfanner N: The protein import machinery of mitochondria. J Biol Chem, 2004;279(15):14473-14476.
Wiedemann N, Truscott KN, Pfannschmidt S, Guiard B, Meisinger C, Pfanner N: Biogenesis of the protein import channel Tom40 of the mitochondrial outer membrane: intermembrane space components are involved in an early stage of the assembly pathway. J Biol Chem, 2004;279(18):18188-18194.
Wiedemann N, Frazier AE, Pfanner N: The protein import machinery of mitochondria. J Biol Chem, 2004;279(15):14473-14476.

2003

Kozjak V, Wiedemann N, Milenkovic D, Lohaus C, Meyer HE, Guiard B, Meisinger C, Pfanner N: An essential role of Sam50 in the protein sorting and assembly machinery of the mitochondrial outer membrane. J Biol Chem, 2003;278(49):48520-48523.
Wiedemann N, Kozjak V, Chacinska A, Schönfisch B, Rospert S, Ryan MT, Pfanner N, Meisinger C: Machinery for protein sorting and assembly in the mitochondrial outer membrane. Nature, 2003;424(6948):565-571.
Wiedemann N, Kozjak V, Prinz T, Ryan MT, Meisinger C, Pfanner N, Truscott KN: Biogenesis of yeast mitochondrial cytochrome c: a unique relationship to the TOM machinery. J Mol Biol, 2003;327(2):465-474.

2002

Geissler A, Chacinska A, Truscott KN, Wiedemann N, Brandner K, Sickmann A, Meyer HE, Meisinger C, Pfanner N, Rehling P: The mitochondrial presequence translocase: an essential role of Tim50 in directing preproteins to the import channel. Cell, 2002;111(4):507-518.
Pfanner N, Wiedemann N: Mitochondrial protein import: two membranes, three translocases. Curr Opin Cell Biol, 2002;14(4):400-411.
Truscott KN, Wiedemann N, Rehling P, Müller H, Meisinger C, Pfanner N, Guiard B: Mitochondrial import of the ADP/ATP carrier: the essential TIM complex of the intermembrane space is required for precursor release from the TOM complex. Mol Cell Biol, 2002;22(22):7780-7789.

2001

Model K, Meisinger C, Prinz T, Wiedemann N, Truscott KN, Pfanner N, Ryan MT: Multistep assembly of the protein import channel of the mitochondrial outer membrane. Nat Struct Biol, 2001;8(4):361-370.
Rehling P, Wiedemann N, Pfanner N, Truscott KN: The mitochondrial import machinery for preproteins. Crit Rev Biochem Mol, 2001;36(3):291-336.
Wiedemann N, Pfanner N, Ryan MT: The three modules of ADP/ATP carrier cooperate in receptor recruitment and translocation into mitochondria. EMBO J, 2001;20(5):951-960.

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