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Select item 112029401.

Knockout Mouse Studies Show That Mitochondrial CLPP Peptidase and CLPX Unfoldase Act in Matrix Condensates near IMM, as Fast Stress Response in Protein Assemblies for Transcript Processing, Translation, and Heme Production

Jana Key, Suzana Gispert, Georg Auburger

Genes (Basel) 2024 Jun; 15(6): 694. Published online 2024 May 27. doi: 10.3390/genes15060694

PMCID:: PMC11202940

Abstract Article PDF–1.4M

Select item 109176602.

Genetic determinants of micronucleus formation in vivo

D. J. Adams, B. Barlas, R. E. McIntyre, I. Salguero, L. van der Weyden, A. Barros, J. R. Vicente, N. Karimpour, A. Haider, M. Ranzani, G. Turner, N. A. Thompson, V. Harle, R. Olvera-León, C. D. Robles-Espinoza, A. O. Speak, N. Geisler, W. J. Weninger, S. H. Geyer, J. Hewinson, N. A. Karp, The Sanger Mouse Genetics Project, B. Fu, F. Yang, Z. Kozik, J. Choudhary, L. Yu, M. S. van Ruiten, B. D. Rowland, C. J. Lelliott, M. del Castillo Velasco-Herrera, R. Verstraten, L. Bruckner, A. G. Henssen, M. A. Rooimans, J. de Lange, T. J. Mohun, M. J. Arends, K. A. Kentistou, P. A. Coelho, Y. Zhao, H. Zecchini, J. R. B. Perry, S. P. Jackson, G. Balmus

Nature. 2024; 627(8002): 130–136. Published online 2024 Feb 14. doi: 10.1038/s41586-023-07009-0

PMCID:: PMC10917660

Abstract Article PDF–4.8M

Select item 108867073.

CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation

Jana Key, Suzana Gispert, Arvind Reddy Kandi, Daniela Heinz, Andrea Hamann, Heinz D. Osiewacz, David Meierhofer, Georg Auburger

Biomolecules. 2024 Feb; 14(2): 241. Published online 2024 Feb 19. doi: 10.3390/biom14020241

PMCID:: PMC10886707

Abstract Article PDF–2.8M

Select item 108554064.

Mitochondrial Unfolded Protein Response Gene Clpp Is Required for Oocyte Function and Female Fertility

Yagmur Ergun, Aysegul Gizem Imamoglu, Mauro Cozzolino, Cem Demirkiran, Murat Basar, Akanksha Garg, Raziye Melike Yildirim, Emre Seli

Int J Mol Sci. 2024 Feb; 25(3): 1866. Published online 2024 Feb 3. doi: 10.3390/ijms25031866

PMCID:: PMC10855406

Abstract Article PDF–1.4M

Select item 107434725.

Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling

Jana Key, Suzana Gispert, Gabriele Koepf, Julia Steinhoff-Wagner, Marina Reichlmeir, Georg Auburger

Int J Mol Sci. 2023 Dec; 24(24): 17503. Published online 2023 Dec 15. doi: 10.3390/ijms242417503

PMCID:: PMC10743472

Abstract Article PDF–6.8M

Select item 105560076.

ClpP/ClpX deficiency impairs mitochondrial functions and mTORC1 signaling during spermatogenesis

Chenxi Guo, Yuan Xiao, Jingkai Gu, Peikun Zhao, Zhe Hu, Jiahuan Zheng, Renwu Hua, Zhuo Hai, Jiaping Su, Jian V. Zhang, William S. B. Yeung, Tianren Wang

Commun Biol. 2023; 6: 1012. Published online 2023 Oct 5. doi: 10.1038/s42003-023-05372-2

PMCID:: PMC10556007

Abstract Article PDF–15M

Select item 99682977.

Ssu72 phosphatase is essential for thermogenic adaptation by regulating cytosolic translation

Eun-Ji Park, Hyun-Soo Kim, Do-Hyoung Lee, Su-Min Kim, Joon-Sup Yoon, Ji-Min Lee, Se Jin Im, Ho Lee, Min-Woo Lee, Chang-Woo Lee

Nat Commun. 2023; 14: 1097. Published online 2023 Feb 25. doi: 10.1038/s41467-023-36836-y

PMCID:: PMC9968297

Abstract Article PDF–4.4M

Select item 98182308.

CLPP Depletion Causes Diplotene Arrest; Underlying Testis Mitochondrial Dysfunction Occurs with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2

Jana Key, Suzana Gispert, Lieke Koornneef, Esther Sleddens-Linkels, Aneesha Kohli, Sylvia Torres-Odio, Gabriele Koepf, Shady Amr, Marina Reichlmeir, Patrick N. Harter, Andrew Phillip West, Christian Münch, Willy M. Baarends, Georg Auburger

Cells. 2023 Jan; 12(1): 52. Published online 2022 Dec 22. doi: 10.3390/cells12010052

PMCID:: PMC9818230

Abstract Article PDF–3.4M

Select item 95151449.

Mitochondrial function and intracellular distribution is severely affected in in vitro cultured mouse embryos

Marta Czernik, Dawid Winiarczyk, Silvestre Sampino, Paweł Gręda, Salvatore Parillo, Jacek Andrzej Modliński, Pasqualino Loi

Sci Rep. 2022; 12: 16152. Published online 2022 Sep 27. doi: 10.1038/s41598-022-20374-6

Correction in:: Sci Rep. 2022; 12: 21276.

PMCID:: PMC9515144

Abstract Article PDF–1.6M

Select item 932773710.

Placental structure, function, and mitochondrial phenotype relate to fetal size in each fetal sex in mice

Esteban Salazar-Petres, Daniela Pereira-Carvalho, Jorge Lopez-Tello, Amanda Nancy Sferruzzi-Perri

Biol Reprod. 2022 Jun; 106(6): 1292–1311. Published online 2022 Mar 16. doi: 10.1093/biolre/ioac056

PMCID:: PMC9327737

Abstract Article PDF–3.0M

Select item 925953311.

KPNB1 modulates the Machado–Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP

Mahkameh Abeditashi, Jonasz Jeremiasz Weber, Priscila Pereira Sena, Ana Velic, Maria Kalimeri, Rana Dilara Incebacak Eltemur, Jana Schmidt, Jeannette Hübener-Schmid, Stefan Hauser, Boris Macek, Olaf Riess, Thorsten Schmidt

Cell Mol Life Sci. 2022 Aug; 79(8): 401. Published online 2022 Jul 6. doi: 10.1007/s00018-022-04372-5

PMCID:: PMC9259533

Abstract Article PDF–4.5M

Select item 898611212.

FGF21 modulates mitochondrial stress response in cardiomyocytes only under mild mitochondrial dysfunction

Marijana Croon, Karolina Szczepanowska, Milica Popovic, Christina Lienkamp, Katharina Senft, Christoph Paul Brandscheid, Theresa Bock, Leoni Gnatzy-Feik, Artem Ashurov, Richard James Acton, Harshita Kaul, Claire Pujol, Stephan Rosenkranz, Marcus Krüger, Aleksandra Trifunovic

Sci Adv. 2022 Apr; 8(14): eabn7105. Published online 2022 Apr 6. doi: 10.1126/sciadv.abn7105

PMCID:: PMC8986112

Abstract Article PDF–2.9M

Select item 873323213.

The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals

Xiaoqiang Sheng, Chuanming Liu, Guijun Yan, Guangyu Li, Jingyu Liu, Yanjun Yang, Shiyuan Li, Zhongxun Li, Jidong Zhou, Xin Zhen, Yang Zhang, Zhenyu Diao, Yali Hu, Chuanhai Fu, Bin Yao, Chaojun Li, Yu Cao, Bin Lu, Zhongzhou Yang, Yingying Qin, Haixiang Sun, Lijun Ding

EBioMedicine. 2022 Jan; 75: 103790. Published online 2021 Dec 30. doi: 10.1016/j.ebiom.2021.103790

PMCID:: PMC8733232

Abstract Article PDF–4.2M

Select item 869911914.

Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA

Jana Key, Sylvia Torres-Odio, Nina C. Bach, Suzana Gispert, Gabriele Koepf, Marina Reichlmeir, A. Phillip West, Holger Prokisch, Peter Freisinger, William G. Newman, Stavit Shalev, Stephan A. Sieber, Ilka Wittig, Georg Auburger

Cells. 2021 Dec; 10(12): 3354. Published online 2021 Nov 29. doi: 10.3390/cells10123354

PMCID:: PMC8699119

Abstract Article PDF–31M

Select item 802670715.

Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA–cGAS–STING Signaling Axis

Sylvia Torres-Odio, Yuanjiu Lei, Suzana Gispert, Antonia Maletzko, Jana Key, Saeed S. Menissy, Ilka Wittig, Georg Auburger, A. Phillip West

J Immunol. Author manuscript; available in PMC 2022 Apr 15.

Published in final edited form as: J Immunol. 2021 Apr 15; 206(8): 1890–1900. Published online 2021 Mar 17. doi: 10.4049/jimmunol.2001016

PMCID:: PMC8026707

Abstract Article PDF–2.4M

Select item 801499816.

The Mouse Heart Mitochondria N Terminome Provides Insights into ClpXP-Mediated Proteolysis

Eduard Hofsetz, Fatih Demir, Karolina Szczepanowska, Alexandra Kukat, Jayachandran N. Kizhakkedathu, Aleksandra Trifunovic, Pitter F. Huesgen

Mol Cell Proteomics. 2020 Aug; 19(8): 1330–1345. Published online 2020 Nov 23. doi: 10.1074/mcp.RA120.002082

PMCID:: PMC8014998

Abstract Article PDF–1.6M

Select item 728320317.

Loss of mitochondrial ClpP, Lonp1, and Tfam triggers transcriptional induction of Rnf213, a susceptibility factor for moyamoya disease

Jana Key, Antonia Maletzko, Aneesha Kohli, Suzana Gispert, Sylvia Torres-Odio, Ilka Wittig, Juliana Heidler, Clea Bárcena, Carlos López-Otín, Yuanjiu Lei, A. Phillip West, Christian Münch, Georg Auburger

Neurogenetics. 2020; 21(3): 187–203. Published online 2020 Apr 28. doi: 10.1007/s10048-020-00609-2

PMCID:: PMC7283203

Abstract Article PDF–1.2M

Select item 711809918.

A salvage pathway maintains highly functional respiratory complex I

Karolina Szczepanowska, Katharina Senft, Juliana Heidler, Marija Herholz, Alexandra Kukat, Michaela Nicole Höhne, Eduard Hofsetz, Christina Becker, Sophie Kaspar, Heiko Giese, Klaus Zwicker, Sergio Guerrero-Castillo, Linda Baumann, Johanna Kauppila, Anastasia Rumyantseva, Stefan Müller, Christian K. Frese, Ulrich Brandt, Jan Riemer, Ilka Wittig, Aleksandra Trifunovic

Nat Commun. 2020; 11: 1643. Published online 2020 Apr 2. doi: 10.1038/s41467-020-15467-7

PMCID:: PMC7118099

Abstract Article PDF–2.1M

Select item 710858719.

Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors

Suzana Gispert, Dajana Parganlija, Michael Klinkenberg, Stefan Dröse, Ilka Wittig, Michel Mittelbronn, Pawel Grzmil, Sebastian Koob, Andrea Hamann, Michael Walter, Finja Büchel, Thure Adler, Martin Hrabé de Angelis, Dirk H. Busch, Andreas Zell, Andreas S. Reichert, Ulrich Brandt, Heinz D. Osiewacz, Marina Jendrach, Georg Auburger

Hum Mol Genet. 2013 Dec 15; 22(24): 4871–4887. Published online 2013 Jul 12. doi: 10.1093/hmg/ddt338

PMCID:: PMC7108587

Abstract Article PDF–896K

Select item 653142620.

Alpha-synuclein suppresses mitochondrial protease ClpP to trigger mitochondrial oxidative damage and neurotoxicity

Di Hu, Xiaoyan Sun, Xudong Liao, Xinwen Zhang, Sara Zarabi, Aaron Schimmer, Yuning Hong, Christopher Ford, Yu Luo, Xin Qi

Acta Neuropathol. 2019; 137(6): 939–960. Published online 2019 Mar 15. doi: 10.1007/s00401-019-01993-2

PMCID:: PMC6531426

Abstract Article PDF–3.1M

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