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.

Autor: Key J; Experimental Neurology, Clinic of Neurology, University Hospital, Goethe University Frankfurt, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany., Gispert S; Experimental Neurology, Clinic of Neurology, University Hospital, Goethe University Frankfurt, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany., Auburger G; Experimental Neurology, Clinic of Neurology, University Hospital, Goethe University Frankfurt, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany.

Publikováno v: Genes [Genes (Basel)] 2024 May 27; Vol. 15 (6). Date of Electronic Publication: 2024 May 27.

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

Autor: Key J; Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany., Gispert S; Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany., Koepf G; Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany., Steinhoff-Wagner J; TUM School of Life Sciences, Animal Nutrition and Metabolism, Technical University of Munich, Liesel-Beckmann-Str. 2, 85354 Freising-Weihenstephan, Germany., Reichlmeir M; Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany., Auburger G; Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany.

Publikováno v: International journal of molecular sciences [Int J Mol Sci] 2023 Dec 15; Vol. 24 (24). Date of Electronic Publication: 2023 Dec 15.

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

Autor: Key J; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany., Gispert S; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany., Koornneef L; Department of Developmental Biology, Erasmus Medical Center, 3015 GD Rotterdam, The Netherlands.; Oncode Institute, Erasmus Medical Center, 3015 GD Rotterdam, The Netherlands., Sleddens-Linkels E; Department of Developmental Biology, Erasmus Medical Center, 3015 GD Rotterdam, The Netherlands., Kohli A; Institute of Biochemistry II, Goethe University Medical School, 60590 Frankfurt am Main, Germany., Torres-Odio S; Department of Microbial Pathogenesis and Immunology, College of Medicine, Health Science Center, Texas A&M University, Bryan, TX 77807, USA., Koepf G; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany., Amr S; Institute of Biochemistry II, Goethe University Medical School, 60590 Frankfurt am Main, Germany., Reichlmeir M; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany., Harter PN; Institute of Neurology (Edinger-Institute), University Hospital Frankfurt, Goethe University, Heinrich-Hoffmann-Strasse 7, 60528 Frankfurt am Main, Germany., West AP; Department of Microbial Pathogenesis and Immunology, College of Medicine, Health Science Center, Texas A&M University, Bryan, TX 77807, USA., Münch C; Institute of Biochemistry II, Goethe University Medical School, 60590 Frankfurt am Main, Germany.; Frankfurt Cancer Institute, 60590 Frankfurt am Main, Germany.; Cardio-Pulmonary Institute, 35392 Gießen, Germany., Baarends WM; Department of Developmental Biology, Erasmus Medical Center, 3015 GD Rotterdam, The Netherlands., Auburger G; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany.

Publikováno v: Cells [Cells] 2022 Dec 22; Vol. 12 (1). Date of Electronic Publication: 2022 Dec 22.

The Bacterial ClpXP-ClpB Family Is Enriched with RNA-Binding Protein Complexes.

Autor: Auburger G; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany., Key J; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany., Gispert S; Experimental Neurology, Medical Faculty, Goethe University, 60590 Frankfurt am Main, Germany.

Publikováno v: Cells [Cells] 2022 Aug 02; Vol. 11 (15). Date of Electronic Publication: 2022 Aug 02.

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

Autor: Key J; Experimental Neurology, Faculty of Medicine, Goethe University, 60590 Frankfurt, Germany., Torres-Odio S; Experimental Neurology, Faculty of Medicine, Goethe University, 60590 Frankfurt, Germany.; Department of Microbial Pathogenesis and Immunology, College of Medicine, Health Science Center, Texas A&M University, Bryan, TX 77807, USA., Bach NC; Center for Protein Assemblies, Chair of Organic Chemistry II, Technical University of Munich, Ernst-Otto-Fischer-Strasse 8, 85748 Garching, Germany., Gispert S; Experimental Neurology, Faculty of Medicine, Goethe University, 60590 Frankfurt, Germany., Koepf G; Experimental Neurology, Faculty of Medicine, Goethe University, 60590 Frankfurt, Germany., Reichlmeir M; Experimental Neurology, Faculty of Medicine, Goethe University, 60590 Frankfurt, Germany.; Faculty of Biosciences, Geothe University, Altenhöferallee 1, 60438 Frankfurt am Main, Germany., West AP; Department of Microbial Pathogenesis and Immunology, College of Medicine, Health Science Center, Texas A&M University, Bryan, TX 77807, USA., Prokisch H; Institute of Neurogenomics, Helmholtz Zentrum München, 85764 Neuherberg, Germany., Freisinger P; Department of Paediatrics, Kreisklinikum Reutlingen, 72764 Reutlingen, Germany., Newman WG; Division of Evolution Infection and Genomics, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester M13 9PL, UK.; Manchester Centre for Genomic Medicine, St Mary's Hospital, Manchester University NHS Foundation Trust, Manchester M13 9PL, UK., Shalev S; Genetic Institute, Emek Medical Center, Afula, Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 3200003, Israel., Sieber SA; Center for Protein Assemblies, Chair of Organic Chemistry II, Technical University of Munich, Ernst-Otto-Fischer-Strasse 8, 85748 Garching, Germany., Wittig I; Functional Proteomics, Institute for Cardiovascular Physiology, Medical Faculty, Goethe University, 60596 Frankfurt am Main, Germany.; German Center for Cardiovascular Research (DZHK), Partner Site RheinMain, 60596 Frankfurt am Main, Germany., Auburger G; Experimental Neurology, Faculty of Medicine, Goethe University, 60590 Frankfurt, Germany.

Publikováno v: Cells [Cells] 2021 Nov 29; Vol. 10 (12). Date of Electronic Publication: 2021 Nov 29.