Search Results - "Key, Jana"

Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling by Key, Jana, Gispert, Suzana, Koepf, Gabriele, Steinhoff-Wagner, Julia, Reichlmeir, Marina, Auburger, Georg

“…The mitochondrial matrix peptidase CLPP is crucial during cell stress. Its loss causes Perrault syndrome type 3 (PRLTS3) with infertility, neurodegeneration,…”
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The Bacterial ClpXP-ClpB Family Is Enriched with RNA-Binding Protein Complexes by Auburger, Georg, Key, Jana, Gispert, Suzana

“…In the matrix of bacteria/mitochondria/chloroplasts, Lon acts as the degradation machine for soluble proteins. In stress periods, however, proteostasis and…”
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Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression by Canet-Pons, Júlia, Sen, Nesli-Ece, Arsović, Aleksandar, Almaguer-Mederos, Luis-Enrique, Halbach, Melanie V., Key, Jana, Döring, Claudia, Kerksiek, Anja, Picchiarelli, Gina, Cassel, Raphaelle, René, Frédérique, Dieterlé, Stéphane, Fuchs, Nina V., König, Renate, Dupuis, Luc, Lütjohann, Dieter, Gispert, Suzana, Auburger, Georg

“…Large polyglutamine expansions in Ataxin-2 (ATXN2) cause multi-system nervous atrophy in Spinocerebellar Ataxia type 2 (SCA2). Intermediate size expansions…”
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Mid-Gestation lethality of Atxn2l -Ablated Mice by Key, Jana, Harter, Patrick N, Sen, Nesli-Ece, Gradhand, Elise, Auburger, Georg, Gispert, Suzana

“…Depletion of yeast/fly Ataxin-2 rescues TDP-43 overexpression toxicity. In mouse models of Amyotrophic Lateral Sclerosis via TDP-43 overexpression, depletion…”
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Progression of pathology in PINK1-deficient mouse brain from splicing via ubiquitination, ER stress, and mitophagy changes to neuroinflammation by Torres-Odio, Sylvia, Key, Jana, Hoepken, Hans-Hermann, Canet-Pons, Júlia, Valek, Lucie, Roller, Bastian, Walter, Michael, Morales-Gordo, Blas, Meierhofer, David, Harter, Patrick N, Mittelbronn, Michel, Tegeder, Irmgard, Gispert, Suzana, Auburger, Georg

“…PINK1 deficiency causes the autosomal recessive PARK6 variant of Parkinson's disease. PINK1 activates ubiquitin by phosphorylation and cooperates with the…”
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Global Proteome of LonP1 +/- Mouse Embryonal Fibroblasts Reveals Impact on Respiratory Chain, but No Interdependence between Eral1 and Mitoribosomes by Key, Jana, Kohli, Aneesha, Bárcena, Clea, López-Otín, Carlos, Heidler, Juliana, Wittig, Ilka, Auburger, Georg

“…Research on healthy aging shows that lifespan reductions are often caused by mitochondrial dysfunction. Thus, it is very interesting that the deletion of…”
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SerThr-PhosphoProteome of Brain from Aged PINK1-KO+A53T-SNCA Mice Reveals pT1928-MAP1B and pS3781-ANK2 Deficits, as Hub between Autophagy and Synapse Changes by Auburger, Georg, Gispert, Suzana, Torres-Odio, Sylvia, Jendrach, Marina, Brehm, Nadine, Canet-Pons, Júlia, Key, Jana, Sen, Nesli-Ece

“…Hereditary Parkinson's disease (PD) can be triggered by an autosomal dominant overdose of alpha-Synuclein (SNCA) as stressor or the autosomal recessive…”
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Increased presence of nuclear DNAJA3 and upregulation of cytosolic STAT1 and of nucleic acid sensors trigger innate immunity in the ClpP-null mouse by Maletzko, Antonia, Key, Jana, Wittig, Ilka, Gispert, Suzana, Koepf, Gabriele, Canet-Pons, Júlia, Torres-Odio, Sylvia, West, A. Phillip, Auburger, Georg

“…Mitochondrial dysfunction may activate innate immunity, e.g. upon abnormal handling of mitochondrial DNA in TFAM mutants or in altered mitophagy. Recent…”
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CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation by Key, Jana, Gispert, Suzana, Kandi, Arvind Reddy, Heinz, Daniela, Hamann, Andrea, Osiewacz, Heinz D, Meierhofer, David, Auburger, Georg

“…The serine peptidase CLPP is conserved among bacteria, chloroplasts, and mitochondria. In humans and mice, its loss causes Perrault syndrome, which presents…”
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Loss of mitochondrial ClpP, Lonp1, and Tfam triggers transcriptional induction of Rnf213, a susceptibility factor for moyamoya disease by Key, Jana, Maletzko, Antonia, Kohli, Aneesha, Gispert, Suzana, Torres-Odio, Sylvia, Wittig, Ilka, Heidler, Juliana, Bárcena, Clea, López-Otín, Carlos, Lei, Yuanjiu, West, A. Phillip, Münch, Christian, Auburger, Georg

“…Human RNF213 , which encodes the protein mysterin, is a known susceptibility gene for moyamoya disease (MMD), a cerebrovascular condition with occlusive…”
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In Cerebellar Atrophy of 12-Month-Old ATM-Null Mice, Transcriptome Upregulations Concern Most Neurotransmission and Neuropeptide Pathways, While Downregulations Affect Prominently Itpr1, Usp2 and Non-Coding RNA by Reichlmeir, Marina, Canet-Pons, Júlia, Koepf, Gabriele, Nurieva, Wasifa, Duecker, Ruth Pia, Doering, Claudia, Abell, Kathryn, Key, Jana, Stokes, Matthew P., Zielen, Stefan, Schubert, Ralf, Ivics, Zoltán, Auburger, Georg

“…The autosomal recessive disorder Ataxia-Telangiectasia is caused by a dysfunction of the stress response protein, ATM. In the nucleus of proliferating cells,…”
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CLPP Depletion Causes Diplotene Arrest; Underlying Testis Mitochondrial Dysfunction Occurs with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2 by Key, Jana, Gispert, Suzana, Koornneef, Lieke, Sleddens-Linkels, Esther, Kohli, Aneesha, Torres-Odio, Sylvia, Koepf, Gabriele, Amr, Shady, Reichlmeir, Marina, Harter, Patrick N, West, Andrew Phillip, Münch, Christian, Baarends, Willy M, Auburger, Georg

“…Human Perrault syndrome (PRLTS) is autosomal, recessively inherited, and characterized by ovarian insufficiency with hearing loss. Among the genetic causes are…”
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Systematic Surveys of Iron Homeostasis Mechanisms Reveal Ferritin Superfamily and Nucleotide Surveillance Regulation to be Modified by PINK1 Absence by Key, Jana, Sen, Nesli Ece, Arsović, Aleksandar, Krämer, Stella, Hülse, Robert, Khan, Natasha Nadeem, Meierhofer, David, Gispert, Suzana, Koepf, Gabriele, Auburger, Georg

“…Iron deprivation activates mitophagy and extends lifespan in nematodes. In patients suffering from Parkinson’s disease (PD), PINK1-PRKN mutations via deficient…”
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Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA by Key, Jana, Torres-Odio, Sylvia, Bach, Nina C, Gispert, Suzana, Koepf, Gabriele, Reichlmeir, Marina, West, A Phillip, Prokisch, Holger, Freisinger, Peter, Newman, William G, Shalev, Stavit, Sieber, Stephan A, Wittig, Ilka, Auburger, Georg

“…Biallelic pathogenic variants in , encoding mitochondrial matrix peptidase ClpP, cause a rare autosomal recessive condition, Perrault syndrome type 3 (PRLTS3)…”
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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 by Key, Jana, Gispert, Suzana, Auburger, Georg

“…LONP1 is the principal AAA+ unfoldase and bulk protease in the mitochondrial matrix, so its deletion causes embryonic lethality. The AAA+ unfoldase CLPX and…”
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Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA–cGAS–STING Signaling Axis by Torres-Odio, Sylvia, Lei, Yuanjiu, Gispert, Suzana, Maletzko, Antonia, Key, Jana, Menissy, Saeed, Wittig, Ilka, Auburger, Georg, West, A. Phillip

“…Abstract Caseinolytic mitochondrial matrix peptidase proteolytic subunit (CLPP) is a serine protease that degrades damaged or misfolded mitochondrial proteins…”
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Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA-cGAS-STING Signaling Axis by Torres-Odio, Sylvia, Lei, Yuanjiu, Gispert, Suzana, Maletzko, Antonia, Key, Jana, Menissy, Saeed S, Wittig, Ilka, Auburger, Georg, West, A Phillip

“…Caseinolytic mitochondrial matrix peptidase proteolytic subunit (CLPP) is a serine protease that degrades damaged or misfolded mitochondrial proteins…”
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