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2024 Original Articles

  • Görgülü I, Jagannath V, Pons S, Koniuszewski F, Groszer M, Maskos U, Huck S, Scholze P (2024) The human‐specific nicotinic receptor subunit CHRFAM7A reduces α7 receptor function in human induced pluripotent stem cells‐derived and transgenic mouse neurons Eur J Neurosci, 60 (5): 4893-4906
  • Müller V, Ernst M, Baykuchkarova A, Koniuszewski F, Bampali K, Seidel T, Scholze P (2024) Probes for the heterogeneity of muscimol binding sites in rat brain Front. Pharmacol., 15: 1368527
  • Golse M, Weinhofer I, Blanco B, Barbier M, Yazbeck E, Huiban C, Chaumette B, Pichon B, Fatemi A, Pascual S, Martinell M, Berger J, Perlbarg V, Galanaud D, Mochel F (2024) Leriglitazone halts disease progression in adult patients with early cerebral adrenoleukodystrophy Brain, 147 (10): 3344-3351
  • Wang J, Kunze M, Villoria-González A, Weinhofer I, Berger J (2024) Peroxisomal Localization of a Truncated HMG-CoA Reductase under Low Cholesterol Conditions Biomol, 14 (2): 244
  • Huber A, Sarne V, Beribisky A, Ackerbauer D, Derdak S, Madritsch S, Etzler J, Huck S, Scholze P, Gorgulu I, Christodoulou J, Studenik C, Neuhaus W, Connor B, Laccone F, Steinkellner H (2024) Generation and Characterization of a Human Neuronal In Vitro Model for Rett Syndrome Using a Direct Reprogramming Method Stem Cells Dev, 33 (5-6): 128-142
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  • Sharmin D, Divović B, Ping X, Cerne R, Smith J, Rezvanian S, Mondal P, Meyer M, Kiley M, Arnold L, Mian M, Pandey K, Jin X, Mitrović J, Djorović D, Lippa A, Cook J, Golani L, Scholze P, Savić M, Witkin J (2024) New Imidazodiazepine Analogue, 5-(8-Bromo-6-(pyridin-2-yl)-4H-benzo-f-imidazo[1,5-a][1,4]diazepin-3-yl)oxazole, Provides a Simplified Synthetic Scheme, High Oral Plasma and Brain Exposures, and Produces Antiseizure Efficacy in Mice, and Antiepileptogenic Activity in Neural Networks in Brain Slices from a Patient with Mesial Temporal Lobe Epilepsy ACS Chem Neurosci, 15 (3): 517-526
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2023 Original Articles

  • Tawbeh A, Raas Q, Tahri-Joutey M, Keime C, Kaiser R, Trompier D, Nasser B, Bellanger E, Dessard M, Hamon Y, Benani A, Di Cara F, Cunha Alves T, Berger J, Weinhofer I, Mandard S, Cherkaoui-Malki M, Andreoletti P, Gondcaille C, Savary S (2023) Immune response of BV-2 microglial cells is impacted by peroxisomal beta-oxidation Front Mol Neurosci, 16: 1299314
  • Villoria-González A, Zierfuss B, Parzer P, Heuböck E, Zujovic V, Waidhofer-Söllner P, Ponleitner M, Rommer P, Göpfert J, Forss-Petter S, Berger J, Weinhofer I (2023) Efficacy of HDAC Inhibitors in Driving Peroxisomal β-Oxidation and Immune Responses in Human Macrophages: Implications for Neuroinflammatory Disorders Biomol, 13 (12): 1696
  • Milica Gajić Bojić, Marco Treven, Kamal P. Pandey, V.V.N. Phani Babu Tiruveedhula, Anja Santrač, Đorđe Đukanović , Nataša Vojinović, Ljiljana Amidžić,Ranko Škrbić, Petra Scholze, Margot Ernst, James M. Cook,Miroslav M. Savić (2023) Vascular effects of midazolam, flumazenil, and a novel imidazobenzodiazepine MP-III-058 on isolated rat aorta Can.J.Physiol.Pharmacol, (19): 2482-2499
  • Werner E, Swinkels D, Juric V, Dorninger F, Baes M, Keller M, Berger J, Watschinger K (2023) Normal plasmalogen levels are maintained in tissues from mice with hepatocyte-specific deletion in peroxin 5 Brain Res Bull, 193: 158-165
  • Weinhofer I, Rommer P, Gleiss A, Ponleitner M, Zierfuss B, Waidhofer-Söllner P, Fourcade S, Grabmeier-Pfistershammer K, Reinert M, Göpfert J, Heine A, Yska H, Casasnovas C, Cantarín V, Bergner C, Mallack E, Forss-Petter S, Aubourg P, Bley A, Engelen M, Eichler F, Lund T, Pujol A, Köhler W, Kühl J, Berger J (2023) Biomarker-based risk prediction for the onset of neuroinflammation in X-linked adrenoleukodystrophy EBioMedicine, 96: 104781
  • Buda A, Forss-Petter S, Hua R, Jaspers Y, Lassnig M, Waidhofer-Söllner P, Kemp S, Kim P, Weinhofer I, Berger J (2023) ABCD1 Transporter Deficiency Results in Altered Cholesterol Homeostasis Biomol, 13 (9): 1333
  • Koniuszewski F, Vogel F, Dajić I, Seidel T, Kunze M, Willeit M, Ernst M (2023) Navigating the complex landscape of benzodiazepine- and Z-drug diversity: insights from comprehensive FDA adverse event reporting system analysis and beyond Front Psychiatry, 14: 1188101
  • Mortensen M, Xu Y, Shehata M, Krall J, Ernst M, Frølund B, Smart T (2023) Pregnenolone sulfate analogues differentially modulate GABA<sub>A</sub> receptor closed/desensitised states Br J Pharmacol,
  • Bampali K, Koniuszewski F, Vogel F, Fabjan J, Andronis C, Lekka E, Virvillis V, Seidel T, Delaunois A, Royer L, Rolf M, Giuliano C, Traebert M, Roussignol G, Fric-Bordat M, Mazelin-Winum L, Bryant S, Langer T, Ernst M (2023) GABAA receptor-mediated seizure liabilities: a mixed-methods screening approach JCB, (6): 2793-2819
  • Martinović K, Bauer J, Kunze M, Berger J, Forss-Petter S (2023) Abcd1 deficiency accelerates cuprizone-induced oligodendrocyte loss and axonopathy in a demyelinating mouse model of X-linked adrenoleukodystrophy Acta Neuropathologica Communications, 11 (1): 98
  • Arias H, De Deurwaerdère P, Scholze P, Sakamoto S, Hamachi I, Di Giovanni G, Chagraoui A (2023) Coronaridine congeners induce sedative and anxiolytic-like activity in naïve and stressed/anxious mice by allosteric mechanisms involving increased GABAA receptor affinity for GABA Eur J Pharmacol, 953: 175854
  • Dorninger F, Kiss A, Rothauer P, Stiglbauer-Tscholakoff A, Kummer S, Fallatah W, Perera-Gonzalez M, Hamza O, König T, Bober M, Cavallé-Garrido T, Braverman N, Forss-Petter S, Pifl C, Bauer J, Bittner R, Helbich T, Podesser B, Todt H, Berger J (2023) Overlapping and Distinct Features of Cardiac Pathology in Inherited Human and Murine Ether Lipid Deficiency Int J Mol Sci, 24 (3): 1884

2023 Reviews

  • Kunze M, Alder N, Hwang I, Roussel G, Karamyshev A (2023) Editorial: Targeting signals in protein trafficking and transport Front Physiol, 14: 1338852
  • Dorninger F, Berger J, Honsho M (2023) Editorial: Solving the plasmalogen puzzle—From basic science to clinical application Front Cell Dev Biol, 11: 1137868

2023 Book Chapters

  • Kunze M (2023) Computational Evaluation of Peroxisomal Targeting Signals in Metazoa. In: , Methods in Molecular Biology Springer: 391-404
  • Bernhard Hochreiter, Johannes A. Schmid, Johannes Berger, Markus Kunze (2023) Estimating the Interaction Strength Between PTS1-Peptides and Their Receptor PEX5 in Living Cells Using Flow-Cytometer-Based FRET (flowFRET) Measurements. In: , Methods in Molecular Biology Springer: 413-434

2022 Original Articles

  • Schmiedhofer P, Vogel F, Koniuszewski F, Ernst M (2022) Cys-loop receptors on cannabinoids: All high? Front Physiol, 13: 1044575
  • Garifulina A, Friesacher T, Stadler M, Zangerl-Plessl E, Ernst M, Stary-Weinzinger A, Willam A, Hering S (2022) β subunits of GABAA receptors form proton-gated chloride channels: Insights into the molecular basis Commun Biol, 5 (1)
  • Zierfuss B, Buda A, Villoria-González A, Logist M, Fabjan J, Parzer P, Battin C, Vandersteene S, Dijkstra I, Waidhofer-Söllner P, Grabmeier-Pfistershammer K, Steinberger P, Kemp S, Forss-Petter S, Berger J, Weinhofer I (2022) Saturated very long-chain fatty acids regulate macrophage plasticity and invasiveness J Neuroinflammation, 19 (1): 305
  • Hochreiter B, Malagon-Vina H, Schmid J, Berger J, Kunze M (2022) Studying the interaction between PEX5 and its full-length cargo proteins in living cells by a novel Försteŕs resonance energy transfer-based competition assay Front Cell Dev Biol, 10: 1026388
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  • Simeone X, Ernst M, Seidel T, Heider J, Enz D, Monticelli S, Vogel F, Koniuszewski F, Langer T, Scholze P, Pace V, Miele M (2022) Novel alpha6 preferring GABA-A receptor ligands based on loreclezole Bioorg Med Chem, 244: 114780
  • Goisis R, Chiavegato A, Gomez-Gonzalo M, Marcon I, Requie L, Scholze P, Carmignoto G, Losi G (2022) GABA tonic currents and glial cells are altered during epileptogenesis in a mouse model of Dravet syndrome Front Cell Neurosci, 16: 1066985
  • Kamoshita M, Kumar R, Anteghini M, Kunze M, Islinger M, Martins dos Santos V, Schrader M (2022) Insights Into the Peroxisomal Protein Inventory of Zebrafish Front Physiol, 13: 822509
  • Weinhofer I, Buda A, Kunze M, Palfi Z, Traunfellner M, Hesse S, Villoria-Gonzalez A, Hofmann J, Hametner S, Regelsberger G, Moser A, Eichler F, Kemp S, Bauer J, Kühl J, Forss-Petter S, Berger J (2022) Peroxisomal very long-chain fatty acid transport is targeted by herpesviruses and the antiviral host response Commun Biol, 5 (1): 944
  • Fallatah W, Cui W, Di Pietro E, Carter G, Pounder B, Dorninger F, Pifl C, Moser A, Berger J, Braverman N (2022) A Pex7 Deficient Mouse Series Correlates Biochemical and Neurobehavioral Markers to Genotype Severity—Implications for the Disease Spectrum of Rhizomelic Chondrodysplasia Punctata Type 1 Front Cell Dev Biol, 10: 886316
  • Dorninger F, Vaz F, Waterham H, Klinken J, Zeitler G, Forss-Petter S, Berger J, Wiesinger C (2022) Ether lipid transfer across the blood-brain and placental barriers does not improve by inactivation of the most abundant ABC transporters Brain Res Bull, 189: 69-79
  • Koniuszewski F, Vogel F, Bampali K, Fabjan J, Seidel T, Scholze P, Schmiedhofer P, Langer T, Ernst M (2022) Molecular Mingling: Multimodal Predictions of Ligand Promiscuity in Pentameric Ligand-Gated Ion Channels MB, 9: 860246
  • Bampali K, Koniuszewski F, Silva L, Rehman S, Vogel F, Seidel T, Scholze P, Zirpel F, Garon A, Langer T, Willeit M, Ernst M (2022) Tricyclic antipsychotics and antidepressants can inhibit α5‐containing GABA <sub>A</sub> receptors by two distinct mechanisms Br J Pharmacol, 0: 1-18
  • Vogel F, Krenn M, Westphal D, Graf E, Wagner M, Leiz S, Koniuszewski F, Augé‐Stock M, Kramer G, Scholze P, Ernst M (2022) A de novo missense variant in GABRA4 alters receptor function in an epileptic and neurodevelopmental phenotype Epilepsia, 0: 1-7

2022 Reviews

  • Dorninger F, Werner E, Berger J, Watschinger K (2022) Regulation of plasmalogen metabolism and traffic in mammals: The fog begins to lift Front Cell Dev Biol, 10: 946393
  • Yifrach E, Rudowitz M, Cruz-Zaragoza L, Tirosh A, Gazi Z, Peleg Y, Kunze M, Eisenstein M, Schliebs W, Schuldiner M, Erdmann R, Zalckvar E (2022) Determining the targeting specificity of the selective peroxisomal targeting factor Pex9 Biol.Chem., 404 (2-3): 121-133
  • Sieghart W, Chiou LC, Ernst M, Fabjan J, M Savić M, Lee MT (2022) α6-Containing GABAA Receptors: Functional Roles and Therapeutic Potentials Pharmacol Rev, 74(1): 238-270

2021 Original Articles

  • Dahan N, Francisco T, Falter C, Rodrigues T, Kalel V, Kunze M, Hansen T, Schliebs W, Erdmann R (2021) Current advances in the function and biogenesis of peroxisomes and their roles in health and disease Histochem Cell Biol, 155 (4): 513-524
  • Rodríguez-Pascau L, Vilalta A, Cerrada M, Traver E, Forss-Petter S, Weinhofer I, Bauer J, Kemp S, Pina G, Pascual S, Meya U, Musolino P, Berger J, Martinell M, Pizcueta P (2021) The brain penetrant PPARγ agonist leriglitazone restores multiple altered pathways in models of X-linked adrenoleukodystrophy Sci Transl Med, 13 (596): eabc0555
  • Fabjan J, Koniuszewski F, Schaar B, Ernst M (2021) Structure-Guided Computational Methods Predict Multiple Distinct Binding Modes for Pyrazoloquinolinones in GABAA Receptors Front Neurosci, 14: 611953
  • Raas Q, van de Beek M, Forss-Petter S, Dijkstra I, Deschiffart A, Freshner B, Stevenson T, Jaspers Y, Nagtzaam L, Wanders R, van Weeghel M, Engelen-Lee J, Engelen M, Eichler F, Berger J, Bonkowsky J, Kemp S (2021) Metabolic rerouting via SCD1 induction impacts X-linked adrenoleukodystrophy J Clin Invest, 131 (8): e142500
  • Weinhofer I, Rommer P, Zierfuss B, Altmann P, Foiani M, Heslegrave A, Zetterberg H, Gleiss A, Musolino P, Gong Y, Forss-Petter S, Berger T, Eichler F, Aubourg P, Köhler W, Berger J (2021) Neurofilament light chain as a potential biomarker for monitoring neurodegeneration in X-linked adrenoleukodystrophy Nat Commun, 12 (1): 1816
  • Simeone X, Koniuszewski F, Müllegger M, Smetka A, Steudle F, Puthenkalam R, Ernst M, Scholze P (2021) A Benzodiazepine Ligand with Improved GABAA Receptor α5-Subunit Selectivity Driven by Interactions with Loop C Mol Pharmacol, 99 (1): 39-48

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