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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
  • 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,
  • 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

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

  • 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
  • Schmiedhofer P, Vogel F, Koniuszewski F, Ernst M (2022) Cys-loop receptors on cannabinoids: All high? Front Physiol, 13
  • 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)
  • Bernhard Hochreiter, Hugo Malagon-Vina, Johannes A. Schmid, Johannes Berger and Markus Kunze (2022) Studying the interaction between PEX5 and its full-length cargo proteins in living cells by a novel Förster´s resonance energy transfer-based competition assay Front Cell Dev Biol,
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  • Eden Yifrach, Markus Rudowitz, Luis Daniel Cruz-Zaragoza, Asa Tirosh , Zohar Gazi , Yoav Peleg , Markus Kunze , Miriam Eisenstein , Wolfgang Schliebs, Maya Schuldiner, Ralf Erdmann and Einat Zalckvar (2022) Determining the targeting specificity of the selective peroxisomal targeting factor Pex9 Biol.Chem.,
  • 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
  • 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
  • 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
  • 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
  • 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

  • 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

  • Fallatah W, Nimmo G, Yergeau C, Di Pietro E, Dorninger F, Berger J, Braverman N (2021) Molecular, biochemical and neuropathological characterization of novel PEX7 deletion variants in patients with classic (severe) rhizomelic chondrodysplasia punctata type 1 Mol Genet Metab, 132: S286
  • 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)
  • 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)
  • 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

2021 Reviews

  • Vilalta, A.; Berger, J; Forss-Petter, S.; Bauer, J.; Weinhofer, I.; Solà, Carme CSIC ORCID; Herrando-Grabulosa, Mireia; Navarro, Xavier; Martinell, M.; Pizcueta, P (2021) Leriglitazone decreases microglia activation and promotes protective phenotypes in several neuroinflammatory models of disease Glia,

2020 Original Articles

  • Dorninger F, Zeitler G, Berger J (2020) Nestlet Shredding and Nest Building Tests to Assess Features of Psychiatric Disorders in Mice BioProtoc., 10 (24)
  • Farr C, El-Kasaby A, Freissmuth M, Sucic S (2020) The Creatine Transporter Unfolded: A Knotty Premise in the Cerebral Creatine Deficiency Syndrome Front.Synaptic Neuroscience, 12
  • Scholze P, Huck S (2020) The α5 Nicotinic Acetylcholine Receptor Subunit Differentially Modulates α4β2* and α3β4* Receptors Front.Synaptic Neuroscience, 12
  • Kumar P, Scholze P, Fronius M, Krasteva‐Christ G, Hollenhorst M (2020) Nicotine stimulates ion transport via metabotropic β4 subunit containing nicotinic ACh receptors Br J Pharmacol, 177 (24): 5595-5608
  • Hochreiter B, Chong C, Hartig A, Maurer-Stroh S, Berger J, Schmid J, Kunze M (2020) A Novel FRET Approach Quantifies the Interaction Strength of Peroxisomal Targeting Signals and Their Receptor in Living Cells Cells, 9 (11): 2381
  • Zierfuss B, Weinhofer I, Buda A, Popitsch N, Hess L, Moos V, Hametner S, Kemp S, Köhler W, Forss‐Petter S, Seiser C, Berger J (2020) Targeting foam cell formation in inflammatory brain diseases by the histone modifier MS‐275 Ann Clin Transl Neurol,
  • Sharp B, Jiang Q, Simeone X, Scholze P (2020) Allosteric Modulation of GABAA Receptors in Rat Basolateral Amygdala Blocks Stress-Enhanced Reacquisition of Nicotine Self-Administration ACS, 3 (6): 1158-1164
  • Scholze P, Pökl M, Längle S, Steudle F, Fabjan J, Ernst M (2020) Two Distinct Populations of α1α6-Containing GABAA-Receptors in Rat Cerebellum Front.Synaptic Neuroscience, 12: 1-10
  • Todt H, Dorninger F, Rothauer P, Fischer C, Schranz M, Bruegger B, Lüchtenborg C, Ebner J, Hilber K, Koenig X, Erdem F, Gawali V, Berger J (2020) Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice J Inherit Metab Dis., 43: 1046-1055
  • Rishi G, Secondes E, Asplett K, Wallace D, Ostini L, Berger J, Subramaniam V (2020) Dysregulated hepcidin response to dietary iron in male mice with reduced Gnpat expression Bio Rep., 40 (8): BSR20201508
  • Dorninger F, Forss-Petter S, Wimmer I, Berger J (2020) Plasmalogens, platelet-activating factor and beyond – Ether lipids in signaling and neurodegeneration Neurobiol Dis, 145: 105061
  • Dalby N, Falk‐Petersen C, Leurs U, Scholze P, Krall J, Frølund B, Wellendorph P (2020) Silencing of spontaneous activity at α4β1/3δ GABA A receptors in hippocampal granule cells reveals different ligand pharmacology Br J Pharmacol, 177 (17): 3975-3990
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  • Zierfuss B, Weinhofer I, Kühl J, Köhler W, Bley A, Zauner K, Binder J, Martinović K, Seiser C, Hertzberg C, Kemp S, Egger G, Leitner G, Bauer J, Wiesinger C, Kunze M, Forss-Petter S, Berger J (2020) Vorinostat in the acute neuroinflammatory form of X-linked adrenoleukodystrophy Ann Clin Transl Neurol, 7 (5): 639-652
  • Perniss A, Latz A, Boseva I, Papadakis T, Dames C, Meisel C, Meisel A, Scholze P, Kummer W, Krasteva-Christ G (2020) Acute nicotine administration stimulates ciliary activity via α3β4 nAChR in the mouse trachea Int. Immunopharmacol., 84: 106496
  • Tsymala I, Nigritinou M, Zeka B, Schulz R, Niederschick F, Matković M, Bauer I, Szalay M, Schanda K, Lerch M, Misu T, Fujihara K, Bennett J, Dahle C, Pache F, Rommer P, Leutmezer F, Illes Z, Leite M, Palace J, Scholze P, Reindl M, Lassmann H, Bradl M (2020) Induction of aquaporin 4-reactive antibodies in Lewis rats immunized with aquaporin 4 mimotopes Acta Neuropathologica Communications, 15; 8 (1)
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  • Werner E, Keller M, Sailer S, Lackner K, Koch J, Hermann M, Coassin S, Golderer G, Werner-Felmayer G, Zoeller R, Hulo N, Berger J, Watschinger K (2020) TheTMEM189gene encodes plasmanylethanolamine desaturase which introduces the characteristic vinyl ether double bond into plasmalogens Proc Natl Acad Sci USA, 117 (14): 7792-7798
  • Arias H, Do Rego J, Do Rego J, Chen Z, Anouar Y, Scholze P, Gonzales E, Huang R, Chagraoui A (2020) Coronaridine congeners potentiate GABAA receptors and induce sedative activity in mice in a benzodiazepine-insensitive manner Prog Neuropsychopharmacol Biol Psychiatry, 101: 109930
  • Steudle F, Rehman S, Bampali K, Simeone X, Rona Z, Hauser E, Schmidt W, Scholze P, Ernst M (2020) A novel de novo variant of GABRA1 causes increased sensitivity for GABA in vitro Sci Rep, 10 (1): 2379
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  • Iorio M, Vogel F, Koniuszewski F, Scholze P, Rehman S, Simeone X, Schnürch M, Mihovilovic M, Ernst M (2020) GABAA Receptor Ligands Often Interact with Binding Sites in the Transmembrane Domain and in the Extracellular Domain—Can the Promiscuity Code Be Cracked? Int J Mol Sci, 21 (1): 334
  • Kunze M (2020) The type-2 peroxisomal targeting signal BBA Mol Cell Res, 1867 (2): 118609

2020 Book Chapters

  • Zarrouk A, Nury T, El Hajj H, Gondcaille C, Andreoletti P, Moreau T, Cherkaoui-Malki M, Berger J, Hammami M, Lizard G, Vejux A (2020) Potential Involvement of Peroxisome in Multiple Sclerosis and Alzheimer’s Disease. In: , Advances in Experimental Medicine and Biology Springer Verlag: 91-104

2019 Original Articles

  • Hochreiter B, Kunze M, Moser B, Schmid J (2019) Advanced FRET normalization allows quantitative analysis of protein interactions including stoichiometries and relative affinities in living cells Sci Rep, 9 (1): 8233
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  • Honsho M, Dorninger F, Abe Y, Setoyama D, Ohgi R, Uchiumi T, Kang D, Berger J, Fujiki Y (2019) Impaired plasmalogen synthesis dysregulates liver X receptor-dependent transcription in cerebellum J. Biochem, 166 (4): 353-361
  • Chong* , Kunze* , Hochreiter , Krenn , Berger , Maurer-Stroh (*equal contribution) (2019) Rare Human Missense Variants can affect the Function of Disease-Relevant Proteins by Loss and Gain of Peroxisomal Targeting Motifs Int J Mol Sci, 20 (18): 4609
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  • Dorninger F, Gundacker A, Zeitler G, Pollak D, Berger J (2019) Ether Lipid Deficiency in Mice Produces a Complex Behavioral Phenotype Mimicking Aspects of Human Psychiatric Disorders Int J Mol Sci, 20 (16): 3929
  • Maleeva G, Wutz D, Rustler K, Nin‐Hill A, Alfonso‐Prieto M, Petukhova E, Bautista‐Barrufet A, Gomila‐Juaneda A, Scholze P, Peiretti F, Rovira C, König B, Gorostiza P, Bregestovski P (2019) A photoswitchable GABA receptor channel blocker Br J Pharmacol, 176(15): 2661-2677
  • Simeone X, Karch R, Ciuraszkiewicz A, Orr‐Urtreger A, Lemmens‐Gruber R, Scholze P, Huck S (2019) The role of the nAChR subunit α5, β2, and β4 on synaptic transmission in the mouse superior cervical ganglion Phys Reports, 7 (6): e14023
  • Dorninger F, König T, Scholze P, Berger M, Zeitler G, Wiesinger C, Gundacker A, Pollak D, Huck S, Just W, Forss-Petter S, Pifl C, Berger J (2019) Disturbed Neurotransmitter Homeostasis in Ether Lipid Deficiency Hum Mol Genet, 28(12): 2046-2061
  • Vasović D, Divović B, Treven M, Knutson D, Steudle F, Scholze P, Obradović A, Fabjan J, Brković B, Sieghart W, Ernst M, Cook J, Savić M (2019) Trigeminal neuropathic pain development and maintenance in rats are suppressed by a positive modulator of α6 GABA A receptors Eur. J. Pain, 23(5): 973-984
  • Prevot T, Li G, Vidojevic A, Misquitta K, Fee C, Santrac A, Knutson D, Stephen M, Kodali R, Zahn N, Arnold L, Scholze P, Fisher J, Marković B, Banasr M, Cook J, Savic M, Sibille E (2019) Novel Benzodiazepine-Like Ligands with Various Anxiolytic, Antidepressant, or Pro-Cognitive Profiles Mol Psychiatry, 5(2): 84-97

2018 Original Articles

  • Knutson D, Kodali R, Divović B, Treven M, Stephen M, Zahn N, Dobričić V, Huber A, Meirelles M, Verma R, Wimmer L, Witzigmann C, Arnold L, Chiou L, Ernst M, Mihovilovic M, Savić M, Sieghart W, Cook J (2018) Design and Synthesis of Novel Deuterated Ligands Functionally Selective for the γ-Aminobutyric Acid Type A Receptor (GABAAR) α6 Subtype with Improved Metabolic Stability and Enhanced Bioavailability J Med Chem, 61 (6): 2422-2446
  • Chiou L, Lee H, Ernst M, Huang W, Chou J, Chen H, Mouri A, Chen L, Treven M, Mamiya T, Fan P, Knutson D, Witzigmann C, Cook J, Sieghart W, Nabeshima T (2018) Cerebellar α6-subunit-containing GABAAreceptors: a novel therapeutic target for disrupted prepulse inhibition in neuropsychiatric disorders Br J Pharmacol, 175 (12): 2414-2427
  • Fan P, Lai T, Hor C, Lee M, Huang P, Sieghart W, Ernst M, Knutson D, Cook J, Chiou L (2018) The α6 subunit-containing GABAA receptor: A novel drug target for inhibition of trigeminal activation Neuropharmacology, 140: 1-13
  • Ricken G, Schwaiger C, De Simoni D, Pichler V, Lang J, Glatter S, Macher S, Rommer P, Scholze P, Kubista H, Koneczny I, Höftberger R (2018) Detection Methods for Autoantibodies in Suspected Autoimmune Encephalitis Front Neurol, 9: 841
  • Forget B, Scholze P, Langa F, Morel C, Pons S, Mondoloni S, Besson M, Durand-de Cuttoli R, Hay A, Tricoire L, Lambolez B, Mourot A, Faure P, Maskos U (2018) A Human Polymorphism in CHRNA5 Is Linked to Relapse to Nicotine Seeking in Transgenic Rats Curr Biol., 28 (20): 3244-3253.e7
  • Siebert D, Bampali K, Puthenkalam R, Varagic Z, Sarto-Jackson I, Scholze P, Sieghart W, Mihovilovic M, Schnürch M, Ernst M (2018) Engineered Flumazenil Recognition Site Provides Mechanistic Insight Governing Benzodiazepine Modulation in GABAA Receptors ACS Chem Biol, 13: 2040-2047
  • Siebert D, Wieder M, Schlener L, Scholze P, Boresch S, Langer T, Schnürch M, Mihovilovic M, Richter L, Ernst M, Ecker G (2018) SAR-Guided Scoring Function and Mutational Validation Reveal Binding Mode of CGS-8216 at the α1+/γ2- Benzodiazepine Site J Chem Inf Model., 58 (8): 1682-1696
  • Elgarf A, Siebert D, Steudle F, Draxler A, Li G, Huang S, Cook J, Ernst M, Scholze P (2018) Different Benzodiazepines Bind with Distinct Binding Modes to GABAA Receptors ACS Chem Biol, 13 (8): 2033-2039
  • Batinić B, Stanković T, Stephen M, Kodali R, Tiruveedhula V, Li G, Scholze P, Marković B, Obradović A, Ernst M, Cook J, Savić M (2018) Attaining in vivo selectivity of positive modulation of α3βγ2 GABA A receptors in rats: A hard task! Eur Neuropsychopharmacol, 28 (8): 903-914
  • Weinhofer I, Zierfuss B, Hametner S, Wagner M, Popitsch N, Machacek C, Bartolini B, Zlabinger G, Ohradanova-Repic A, Stockinger H, Köhler W, Höftberger R, Regelsberger G, Forss-Petter S, Lassmann H, Berger J (2018) Impaired plasticity of macrophages in X-linked adrenoleukodystrophy Brain, 141: 2329-2342
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  • Dorninger F, Moser A, Kou J, Wiesinger C, Forss-Petter S, Gleiss A, Hinterberger M, Jungwirth S, Fischer P, Berger J (2018) Alterations in the Plasma Levels of Specific Choline Phospholipids in Alzheimer’s Disease Mimic Accelerated Aging J. Alzheimers Dis., 62 (2): 841-854
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  • Treven M, Siebert D, Holzinger R, Bampali K, Fabjan J, Varagic Z, Wimmer L, Steudle F, Scholze P, Schnürch M, Mihovilovic M, Ernst M (2018) Towards Functional Selectivity for α6β3γ2 GABAA Receptors: A Series of Novel Pyrazoloquinolinones Br J Pharmacol, 175 (3): 419-428

2018 Book Chapters

  • Kunze M (2018) Predicting Peroxisomal Targeting Signals to Elucidate the Peroxisomal Proteome of Mammals. In: del Río, Luis A. (Ed.), Subcellular Biochemistry Vol. 69: Peroxisomes and their Key Role in Cellular Signaling and Metabolism Springer Verlag, Heidelberg: 157-199

2017 Original Articles

  • Wongsamitkul N, Maldifassi M, Simeone X, Baur R, Ernst M, Sigel E (2017) α subunits in GABAA receptors are dispensable for GABA and diazepam action Sci Rep, 7 (1)
  • Simeone X, Siebert D, Bampali K, Varagic Z, Treven M, Rehman S, Pyszkowski J, Holzinger R, Steudle F, Scholze P, Mihovilovic M, Schnürch M, Ernst M (2017) Molecular tools for GABAA receptors: High affinity ligands for β1-containing subtypes Sci Rep, 7 (1) : 5674
  • Trigueros-Motos L, van Capelleveen J, Torta F, Castaño D, Zhang LH, Chai C, Kang M, Dimova L, Schimmel A, Tietjen I, Radomski C, Tan L, Hwee T, Narayanaswamy P, Wu D, Dorninger F, Yakala G, Barhdadi A, Angeli V, Dubé M, Berger J, Dallinga-Thie G, Tietge U, Wenk M, Hayden M, Hovingh G, Singaraja R (2017) ABCA8 Regulates Cholesterol Efflux and High-Density Lipoprotein Cholesterol Levels Arterioscler Thromb Vasc Biol, 37: 2147-2155
  • Ghafari M, Falsafi S, Szodorai E, Kim E, Li L, Höger H, Berger J, Fuchs K, Sieghart W, Lubec G (2017) Formation of GABAA receptor complexes containing α1 and α5 subunits is paralleling a multiple T-maze learning task in mice Brain Struct Funct, 222 (1): 549-561
  • Dorninger F, Herbst R, Kravic B, Camurdanoglu B, Macinkovic I, Zeitler G, Forss-Petter S, Strack S, Khan M, Waterham H, Rudolf R, Hashemolhosseini S, Berger J (2017) Reduced Muscle Strength in Ether Lipid-Deficient Mice Is Accompanied by Altered Development and Function of the Neuromuscular Junction J Neurochem, 143: 569-583
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2017 Reviews

  • Dorninger F, Forss-Petter S, Berger J (2017) From peroxisomal disorders to common neurodegenerative diseases - the role of ether phospholipids in the nervous system FEBS Letters, 591: 2761-2788
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2016 Original Articles

  • Puthenkalam R, Hieckel M, Simeone X, Suwattanasophon C, Feldbauer R, Ecker G, Ernst M (2016) Structural Studies of GABAA Receptor Binding Sites: Which Experimental Structure Tells us What? Front Mol Neurosci, 16;9:44
  • Stamenić T, Poe M, Rehman S, Santrač A, Divović B, Scholze P, Ernst M, Cook J, Savić M (2016) Ester to amide substitution improves selectivity, efficacy and kinetic behavior of a benzodiazepine positive modulator of GABAA receptors containing the α5 subunit Eur J Pharmacol, 791: 433-443
  • Mandl P, Hayer S, Karonitsch T, Scholze P, Győri D, Sykoutri D, Blüml S, Mócsai A, Poór G, Huck S, Smolen J, Redlich K (2016) Nicotinic acetylcholine receptors modulate osteoclastogenesis Arthritis Res Ther, 18 (1): 63
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2016 Reviews

  • Berger J, Dorninger F, Forss-Petter S, Kunze M (2016) Peroxisomes in brain development and function Biochim Biophys Acta, 863: 934-955
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2015 Original Articles

  • Kunze M, Berger J (2015) The similarity between N-terminal targeting signals for protein import into different organelles and its evolutionary relevance Front Physiol, 6: 1-27
  • Jung G, Kim E, Cicvaric A, Sase S, Gröger M, Höger H, Sialana F, Berger J, Monje F, Lubec G (2015) Drebrin depletion alters neurotransmitter receptor levels in protein complexes, dendritic spine morphogenesis and memory-related synaptic plasticity in the mouse hippocampus J Neurochem, 134 (2): 327-339
  • Mika J, Schwarz K, Wanzenboeck H, Scholze P, Bertagnolli E (2015) Investigation of neurotrophic factor concentrations with a novel in vitro concept for peripheral nerve regeneration J Neurosci Res, 93 (11): 1631-1640
  • Dorninger F, Wiesinger C, Braverman N, Forss-Petter S, Berger J (2015) Ether Lipid Deficiency Does Not Cause Neutropenia or Leukopenia in Mice and Men Cell Metab, 21 (5): 650-651
  • Xanthos D, Beiersdorf J, Thrun A, Ianosi B, Orr-Urtreger A, Huck S, Scholze P (2015) Role of α5-containing nicotinic receptors in neuropathic pain and response to nicotine Neuropharmacology, 95: 37-49
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  • Kunze M, Malkani N, Maurer-Stroh S, Wiesinger C, Schmid J, Berger J (2015) Mechanistic Insights into PTS2-mediated Peroxisomal Protein Import: The Co-receptor PEX5L drastically increases the Interaction Strength between the Cargo Protein and the Receptor PEX7 J Biol Chem, 290: 4928-4940
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  • Dorninger F, Brodde A, Braverman N.E, Moser A.B, Just W, Forss-Petter S, Brügger B. Berger J, (2015) Homeostasis of Phospholipids – The Level of Phosphatidylethanolamine Tightly Adapts to Changes in Ethanolamine Plasmalogens Biochim Biophys Acta, 1851: 117-128
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  • Ghafari M, Whittle N, Miklósi A, Kotlowsky C, Schmuckermair C, Berger J, Bennett K, Singewald N, Lubec G (2015) Dietary magnesium restriction reduces amygdala–hypothalamic GluN1 receptor complex levels in mice Brain Struct Funct, 220 (4): 2209-2221
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2015 Reviews

  • Wiesinger C, Eichler F, Berger J, (2015) The genetic landscape of X-linked adrenoleukodystrophy: inheritance, mutations, modifier genes, and diagnosis Appl Clin Genet, 8: 109
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2014 Original Articles

  • Muneer Z, Wiesinger C, Voigtländer T, Werner H, Berger J, Forss-Petter S (2014) Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice PLoS One, 9 (9): e108655
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  • Weber F, Weinhofer I, Einwich A, Forss-Petter S, Muneer Z, Maier H, Weber W, Berger J (2014) Evaluation of Retinoids for Induction of the Redundant Gene ABCD2 as an Alternative Treatment Option in X-Linked Adrenoleukodystrophy PLoS One, 9 (7): e103742
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  • Saroja S, Sase A, Kircher S, Wan J, Berger J, Höger H, Pollak A, Lubec G (2014) Hippocampal proteoglycans brevican and versican are linked to spatial memory of Sprague-Dawley rats in the morris water maze J Neurochem, 130 (6): 797-804
  • Beiranvand F, Zlabinger C, Orr-Urtreger A, Ristl R, Huck S, Scholze P (2014) Nicotinic acetylcholine receptors control acetylcholine and noradrenaline release in the rodent habenulo-interpeduncular complex Br J Pharmacol, 171 (23): 5209-5224
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  • Weber F, Wiesinger Ch, Forss-Petter S, Regelsberger G, Einwich A, Weber W, Köhler W, Stockinger H, Berger J, (2014) X-linked adrenoleukodystrophy: Very long-chain fatty acid metabolism is severely impaired in monocytes but not in lymphocytes Hum Mol Genet, 23 (10): 2542-2550
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  • Gondcaille C, C.Genin E, E.Lopez T, MM.Dias A, Geillon F, Andreoletti P, Cherkaoui-Malki M, Nury T, Lizard G, Weinhofer-Molisch I, Berger J, T.Kase E, Trompier D, Savary St, (2014) LXR antagonists induce ABCD2 expression Biochim Biophys Acta, 1841(2): 259-266
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  • Mirheydari P, Ramerstorfer J, Varagic Z, Scholze P, Wimmer L, Mihovilovic MM, Sieghart W, Ernst M (2014) Unexpected properties of delta-containing GABA-A receptors in response to ligands interacting with the alpha+beta-site Neurochem Res, 39 (6): 1057-1067

2014 Reviews

  • Berger J, Forss-Petter S, Eichler F.S, (2014) Pathophysiologie of X-linked adrenoleukodystrophy Biochimie, 98: 135-142
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2014 Book Chapters

  • Kunze M, Berger J (2014) Single Peroxisomal Enzyme and Transporter Deficiencies in Human Diseases and Mouse Models. In: , Molecular Machines Involved in Peroxisome Biogenesis and Maintenance Springer Vienna: pp 153-184
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2013 Original Articles

  • Wang B, Van Veldhoven P, Brees C, Rubio N, Nordgren M, Apanasets O, Kunze M, Baes M, Agostinis P, Fransen M (2013) Mitochondria are targets for peroxisome-derived oxidative stress in cultured mammalian cells FREE RADICAL BIO MED , 65: 882-894
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  • Pei Z, Fraisl P, Shi X, Gabrielson E, Forss-Petter S, Berger J, Watkins P.A (2013) Very Long-Chain Acyl-COA Synthetase 3:Overexpression and Growth Dependence in Lung Cancer PLoS.ONE., 8(7): e69392
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  • Ciuraszkiewicz A, Schreibmayer W, Platzer D, Orr-Urtreger A, Scholze P, Huck S. (2013) Single-channel properties of α3β4, α3β4α5, and α3β4β2 nicotinic ACh receptors in mice lacking specific nAChR subunits J Physiol, 591: 3271-3288
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  • Wiesinger C, Kunze M, Regelsberger G, Forss-Petter S, Berger J. (2013) Impaired Very Long-Chain Acyl-CoA β-Oxidation in Human X-ALD Fibroblasts Is a Direct Consequence of ABCD1 Transporter Dysfunction J Biol Chem, 288: 19269-19279

2013 Reviews

  • Kunze M, Hartig A, (2013) Permeability of the peroxisomal membrane: lessons from the glyoxylate cycle Front Physiol, 4
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2013 Book Chapters

  • Weinhofer I, Kunze M, Forss-Petter S, Berger J (2013) Involvement of Human Peroxisomes in Biosynthesis and Signaling of Steroid and Peptide Hormones. In: del Río, Luis A. (Ed.), Subcellular Biochemistry Vol. 69: Peroxisomes and their Key Role in Cellular Signaling and Metabolism Springer Verlag, Heidelberg: pp 101-110

2012 Original Articles

  • Berger ML, Pöhler T, Schadt O, Stanger M, Rebernik P, Scholze P, Noe CR (2012) Exploring the Polyamine Regulatory Site of the NMDA Receptor: a Parallel Synthesis Approach ChemMedChem, 8 (1): 82-94
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  • Brodde A, Teigler A, Brugger B, Lehmann WD, Wieland F, Berger J, Just WW. (2012) Impaired neurotransmission in ether lipid-deficient nerve terminals Hum Mol Genet, 21(12): 2713-2724.
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  • Preusser M, Hassler M, Birner P, Rudas M, Acker T, Plate H, Widhalm G, Knosp E, Breitschopf H, Berger J, Marosi C. (2012) Microvascularization and expression of VEGF and its receptors in recurring meningiomas: pathobiological data in favor of anti-angiogenic therapy approaches Clin Neuropathol, 31(5): 352-360
  • Scholze P, Koth G, Orr-Urtreger A, Huck S (2012) Subunit composition of α5-containing nicotinic receptors in the rodent habenula J Neurochem, 121(4): 551-560
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  • Facciotti F, Ramanjaneyulu GS, Lepore M, Sansano S, Cavallari M, Kistowska M, Forss-Petter S, Ni G, Colone A, Singhal A, Berger J, Xia C, Mori L, De Libero G (2012) Peroxisome-derived lipids are self-antigens stimulating invariant Natural Killer T cells in the thymus Nature Immunol, 13(5): 474-480.
  • Barbier M, Sabbagh A, Kasper E, Asheuer M, Ahouansou O, Pribill I, Forss-Petter S, Vidaud M, Berger J, Aubourg P (2012) CD1 gene polymorphisms and phenotypic variability in X-linked adrenoleukodystrophy PLoS.ONE., 7(1): e29872
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2012 Reviews

  • Kemp S, Berger J, Aubourg P (2012) X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects BBA, 1822 (9): 1465-1474

2011 Original Articles

  • Wolff P, Weinhofer I, Seguin J, Roszak P, Beisel C, Donoghue MT, Spillane C, Nordborg M, Rehmsmeier M, Köhler C. (2011) High-resolution analysis of parent-of-origin allelic expression in the Arabidopsis Endosperm PLoS Genet, 7(6): e1002126.
  • Kunze M, Neuberger G, Maurer-Stroh S, Ma J, Eck T, Braverman N, Schmid J, Eisenhaber F, Berger J. (2011) Structural requirements for the interaction of peroxisomal targeting signal 2 and its receptor PEX7 J Biol Chem, 286(52): 45048-45062
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  • Kou J, Kovacs GG, Höftberger R, Kulik W, Brodde A, Forss-Petter S, Hönigschnabl S, Gleiss A, Brügger B, Wanders R, Just W, Budka H, Jungwirth S, Fischer P, Berger J. (2011) Peroxisomal alterations in Alzheimer's disease Acta Neuropathol, 122: 271-283
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  • Scholze P, Ciuraszkiewicz A, Grössl F, Orr-Urtreger A, McIntosh JM, Huck S (2011) Alpha4beta2 Nicotinic Acetylcholine Receptors n the Early Postnatal Mouse Superior Cervical Ganglion Dev. Neurobiology, 71 (5): 390-399
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  • Peneder TM, Scholze P, Berger ML, Reither H, Heinze G, Bertl J, Bauer J, Richfield EK, Hornykiewicz O, Pifl C (2011) Chronic exposure to manganese decreases striatal dopamine turnover in human alpha-synuclein transgenic mice Neuroscience, 180: 280-292

2010 Original Articles

  • Anzola JM, Sieberer T, Ortbauer M, Butt H, Korbei B, Weinhofer I, Müllner AE, Luschnig C. (2010) Putative Arabidopsis transcriptional adaptor protein (PROPORZ1) is required to modulate histone acetylation in response to auxin Proc Natl Acad Sci USA, 107(22): 10308-13
  • Weinhofer I, Hehenberger E, Roszak P, Hennig L, Köhler C. (2010) H3K27me3 profiling of the endosperm implies exclusion of polycomb group protein targeting by DNA methylation PLoS Genet, 6(10): e1001152
  • Höftberger R, Kunze M, Voigtländer T, Unterberger U, Regelsberger G, Bauer J, Aboul-Enein F, Garzuly F, Forss-Petter S, Bernheimer H, Berger J, Budka H (2010) Peroxisomal Localization of the Proopiomelanocortin-Derived Peptides β-Lipotropin and β-Endorphin Endocrinology, 151 (10): 4801-4810
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  • Ramerstorfer J, Furtmüller R, Vogel E, Huck S, Sieghart W (2010) The point mutation gamma2F77I changes the potency and efficacy of benzodiazepine site ligands in different GABAA receptor subtypes Eur J Pharmacol, 636: 18-27
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  • David R, Ciuraszkiewicz A, Simeone X, Orr-Urtreger A, Papke RL, McIntosh JM, Huck S, Scholze P (2010) Biochemical and functional properties of distinct nicotinic acetylcholine receptors in the superior cervical ganglion of mice with targeted deletions of nAChR subunit genes Eur J Neurosci, 6: 978-993
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2010 Reviews

  • Köhler C, I. Weinhofer-Molisch (2010) Mechanisms and evolution of genomic imprinting in plants Heredity, 105: 57-63
  • Berger J, Pujol A, Aubourg P, Forss-Petter S (2010) Current and future pharmacological treatment strategies in X-linked adrenoleukodystrophy. Brain Pathol, 20: 845-856
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2009 Original Articles

  • Nicolussi EM, Huck S, Lassmann H, Bradl M (2009) The cholinergic anti-inflammatory system limited T cell infiltration into the neurodegenerative CNS, but can not counteract complex CNS inflammation Neurobiol Dis, 35: 24-31

2008 Original Articles

  • Savic MM, Clayton T, Furtmüller R, Gavrilovic I, Samardzic J, Savic S, Huck S, Sieghart W, Cook JM (2008) PWZ-029, a compound with moderate inverse agonist functional selectivity at GABA-A receptors containing alpha5 subunits, improves passive, but not active, avoidance learning in rats Brain Res, 1208: 150-159
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  • Maier EM, Mayerhofer PU, Asheuer M, Köhler W, Rothe M, Muntau AC, Roscher AA, Holzinger A, Aubourg P, Berger J (2008) X-linked adrenoleukodystrophy phenotype is independent of ABCD2 genotype Biochem Bioph Res Comm, 377: 176-180
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  • Weinhofer I, Kunze M, Rampler H, Forss-Petter S, Samarut J, Plateroti M, Berger J (2008) Distinct modulatory roles for thyroid hormone receptors TR-alpha and TR-beta in SREBP1-activated ABCD2 expression Eur J Cell Biol, 87: 933-945
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  • Furtmüller R, Furtmüller B, Ramerstorfer J, Paladini AC, Wasowski C, Marder M, Huck S, Sieghart W (2008) 6,3'-Dinitroflavone is a low efficacy modulator of GABA(A) receptors Eur J Pharmacol, 591: 142-146
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  • Harris D, Clayton T, Cook J, Sahbaie P, Halliwell RF, Furtmüller R, Huck S, Sieghart W, and DeLorey TM (2008) Selective influence on contextual memory: physiochemical properties associated with selectivity of benzodiazepine ligands at GABAA receptors containing the alpha5 subunit J Med Chem, 51: 3788-3803
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  • Putz G, Kristufek D, Orr-Urtreger A, Changeux JP, Huck S, Scholze P (2008) Nicotinic acetylcholine receptor-subunit mRNAs in the mouse superior cervical ganglion are regulated by development but not by deletion of distinct subunit genes J Neurosci Res, 86: 972-981
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  • Savic MM, Huang S, Furtmüller R, Clayton T, Huck S, Obradovic DI, Ugresic ND, Sieghart W, Bokonjic DR, Cook JM (2008) Are GABA(A) Receptors Containing alpha5 Subunits Contributing to the Sedative Properties of Benzodiazepine Site Agonists? Neuropsychopharm, 33: 332-339
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2007 Original Articles

  • Scholze P., Orr-Urtreger A., Changeux J.-P., McIntosh J.M. , Huck S (2007) Catecholamine outflow from mouse and rat brain slice preparations evoked by nicotinic acetylcholine receptor activation and electrical field stimulation Br J Pharmacol, 151: 414-422
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  • Sarto-Jackson I, Furtmüller R, Ernst M, Huck S, Sieghart W (2007) Spontaneous cross-link of mutated alpha1 subunits during GABA-A receptor assembly J Biol Chem, 282: 4354-4363
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  • Wiese S, Gronemeyer T, Ofmann R, Kunze M, Grou CP, Almeida JA, Eisenacher M, Stephan C, Hayen H, Pawlas M, Bunse C, Schollenberger L, Korosec T, Waterham HR, Schliebs W, Erdmann R, Berger J et al (2007) Proteomic characterization of mouse kidney peroxiosmes by tandem mass spectrometry and protein correlation profiling Mol Cell Proteomics, 6: 2045-2057
  • Höftberger R, Kunze M, Weinhofer I, Aboul-Enein F, Voigtländer T, Oezen I, Amann G, Bernheimer H, Budka H, Berger J (2007) Distribution and cellular localization of adrenoleukodystrophy protein in human tissues: Implications for X-linked adrenoleukodystrophy Neurobiol Dis, 28 (2): 165-174
  • Barceló-Coblijn G., Golkovko MY., Weinhofer I., Berger J., Murphy E.J. (2007) Brain neutral lipids mass is increased in a-synuclein gene ablated mice J Neurochem, 101: 132-141
  • Dumser M, Bauer J, Lassmann H, Berger J, Forss-Petter S (2007) Lack of adrenoleukodystrophy protein enhances oligodendrocyte disturbance and microglia activation in mice with combined Abcd1/Mag deficiency Acta Neuropathol, 114;6: 573-586

2006 Original Articles

  • Golovko Y.M., Rosenberger T.A., Fargeman N.J., Feddersen S., Cole N.B, Pribill I., Berger J., Nussbaum R.L., Murphy E.J (2006) Acyl-CoA Synthetase activity links wild-type but not mutant alpha-synuclein to brain arachidonate metabolism Biochemistry, 45: 6956-6966
  • Weinhofer I., Kunze M., Stangl H., Porter F.D., Berger J (2006) Peroxisomal cholesterol biosynthesis and Smith-Lemli-Opitz syndrome. Biochem Bioph Res Comm, 345: 205-209
  • Fraisl P., Tanaka H., Forss-Petter S, Lassmann H., Nishimune Y., Berger J (2006) A novel mammalian bubblegum-related acyl-CoA synthetase restricted to testes and possibly involved in spermatogenesis Arch Biochem Biophys, 451: 23-33
  • Yang J-W., Afjehi-Sadat L., Gelpi E., Kunze M., Höger H., Fleckner J., Berger J., Lubec G (2006) Proteom Profiling in the rat Harderian gland J Proteome Res, 5: 1751-1762
  • Rauschka H., Colsch B., Baumann N., Wevers R., Schmidbauer M., Krammer M., Turpin J.C., Lefevre M., Olivier C., Tardieu S., Krivit W., Moser H., Moser A., Gieselmann V,.....Berger J (2006) Late onset metachromatic leukodystrophy: genotype strongly influences phenotype Neurology, 67: 859-863
  • Hochmeister S., Grundtner R., Bauer J., Engelhardt B., Lyck R., Gordon G., Korosec T., Kutzelnigg A., Berger J., Bradl M., Bittner R.E., Lassmann H (2006) Dysferlin Is a New Marker for Leaky Brain Blood Vessels in Multiple Sclerosis J Neuropath Exp Neurol, 65: 855-865

2006 Reviews

  • Berger J, Gärtner J (2006) X-linked adrenoleukodystrophy: Clinical, biochemical and pathogenetic aspects BBA Mol Cell Res, 1763: 1721-1732
  • Kunze M, Pracharoentwattana I, Smith SM, Hartig A (2006) A central role for the peroxisomal membrane in glyoxylate cycle function BBA Mol Cell Res, 1763: 1441-1452

2005 Original Articles

  • Fischer H., Orr-Urtreger A., Role L.W., Huck S (2005) Selective deletion of the alpha 5 subunit differentially affects somatio-dendritic versus axonally targeted nicotinic ACh receptors in mouse J Physiol, 563: 119-137
  • DiRusso C. C., Li H, Drwis D, Watkins P, Berger J., Black P.N (2005) Comparative biochemical studies of the murine fatty acid transport proteins (FATP) expressed in yeast J Biol Chem, 280: 16829-16837
  • Oezen I., Rossmanith W., Forss-Petter S., Kemp S., Voigtländer T., Moser-Thier., Wanders R., Bittner R., Berger J (2005) Accumulation of very long-chain fatty acids does not affect mitochondrial function in Adrneoleukodystrophy protein deficiency Hum Mol Genet, 14: 1127-1137
  • Lugowska A., Berger J., Tylki-Szymanska A., Löschl B., Molzer B., Zobel M., Czartoryska B (2005) Molecular and phenotypic characteristics of metachromatic leukodystrophy patients from Poland Clin Genet, 68: 48-54
  • Weinhofer I., Forss-Petter S., Kunze M., Zigman M., Berger J. (2005) X-linked adrenoleukodystrophy mice demonstrate abnormalities in cholesterol metabolism FEBS Letters, 579: 5512-5516
  • Lugowska A., Amaral O., Berger J., Berna L., Bosshard N.U., Chabas A., Fensom A., Gieselmann V., Gorovenko NG., Lissens W., Mansson J.E., Marcap A., Michelakakis H., Bernheimer H., Olkhovych N.V. et a (2005) Mutations c.459+1G>A and p.P426L in the ARSA gene: prevalence in metachromatic leukodystrophy patients from European countries Mol Genet Metab, 86: 353-359
  • Weinhofer I., Kunze M., Rampler H., Bookout A.L., Forss-Petter S., Berger J (2005) LXRalpha interferes with SREBP1c-mediated Abcd2 expression: novel cross-talk in gene regulation J Biol Chem, 280: 41243-41251

2004 Original Articles

  • Fraisl P., Forss-Petter S., Zigman M., Berger J. (2004) Murine bubblegum orthologue is a microsomal very long-chain acyl-CoA synthetase Biochemistry, 377: 85-93
  • Mashek D.G., Bornfeldt K.E., Coleman R.A., Berger J., Bernlohr D.A., Black P., DiRusso C.C., Farber S.A., Guo W., Hashimoto N., Khodiyar V., Kuypers F.A., Maltais L. J., Nebert D.W., Renieri A., S (2004) Revised nomenclature for the mammalian long chain acyl-CoA synthetase (ACS) gene family J Lipid Res, 45: 1958-1961
  • Birner P., Preusser M., Gelpi E., Berger J., Gatterbauer B., Ambros I.M., Ambros P.F., Acker T., Plate K.H., Harris A.L., Hainfellner J.A (2004) Expression of hypoxia-related tissue factors correlates with diminished survival of adjuvantly treated patients with chromosome 1p-aberrant oligodendroglial neoplasms: therapeutic implications Clin Cancer Res, 10: 6567-6571
  • Pei Z., Fraisl P., Berger J., Jia Z., Forss-Petter S., Watkins P (2004) Mouse very long-chain acyl-CoA synthetase 3/Fatty Acid Transporter Protein 3 Catalyzes fatty acid activation but not fatty acid transport in MA-10 cells J Biol Chem, 279: 54454-54462
  • Neuberger G., Kunze M., Eisenhaber F., Berger J., Hartig A., Brocard C. (2004) Hidden localisation motifs: Naturally occurring peroxisomal targeting signals in non-peroxisomal proteins Genome Biol, 5: R97

2003 Original Articles

  • Weinhofer I, Forss-Petter S, Zigman M, Berger J (2003) Cholesterol regulates ABCD2 gene expression: implications for X-linked adrenoleukodstrophy Adv Exp Med Biol, 544: 331-332
  • Li X, Cao H, Zhang C, Furtmüller R, Fuchs K, Huck S, Sieghart W, Deschamps J, Cook JM (2003) Synthesis, in vitro affinity, and efficacy of a bis 8-ethynyl-4H-imidazol[1,5a]- [1,4]benzo-diazepine analogue, the first bivalent alpha5 subtype selective BzR/GABAA antagonist J Med Chem, 46: 5567-5570
  • Lassmann H., Reindl M., Rauschka H., Berger J., Aboul-Enein F., Berger T., Zurbriggen A., Luterotti A., Brück W., Weber JR., Ullrich R., Schmidbauer M., Jellinger K., Vanddevelde M. (2003) A new paraclinical CSF marker for hypoxia-like tissue damage in multiple sclerosis lesions Brain, 126: 1347-1357
  • Fourcade S., Savary S., Gondcaille C., Berger J., Netik A., Cadepond F., Etr ME., Molzer B., Bugaut M (2003) Thyroid hormone induction of the drenoleukodystrophy-related gene (ABCD2) Mol Pharmacol, 63: 1296-1303
  • Rampler H., Weinhofer I., Netik A., Forss-Petter S., Brown PJ., Oplinger JA., Bugaut M., Berger J (2003) Evaluation of the therapeutic potential of PPAR alpha agonists for X-linked adrenoleukodystrophy. Mol Genet Metab, 80: 398-407

2003 Book Chapters

  • Berger J, Kunze M, Forss-Petter S (2003) Lessons from Knockout Mice II: Mouse Models for Peroxisomal Disorders with Single Protein Deficiency. In: , Advances in Experimental Medicine and Biology Springer Verlag: 123-134

2002 Original Articles

  • Kunze M, Kragler F, Binder M, Hartig A, Gurvitz A (2002) Targeting of malate synthase 1 to the peroxisomes of Saccharomyces cerevisiae cells depends on growth on oleic acid medium Eur J Biochem, 269 (3): 915-922
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  • Scholze P, Freissmuth M, Sitte H (2002) Mutations within an Intramembrane Leucine Heptad Repeat Disrupt Oligomer Formation of the Rat GABA Transporter 1 J Biol Chem, 277 (46): 43682-43690
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  • Scholze P (2002) The Role of Zinc Ions in Reverse Transport Mediated by Monoamine Transporters J Biol Chem, 277 (24): 21505-21513
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  • Sitte H, Singer E, Scholze P (2002) Bi-directional transport of GABA in human embryonic kidney (HEK-293) cells stably expressing the rat GABA transporter GAT-1 Br J Pharmacol, 135 (1): 93-102
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  • Furtmüller R, Schlag MG, Berger M L, Hopf R, Huck S, Sieghart W, Redl H (2002) Tranexamic acid, a widely used antifibrinolytic agent, causes convulsions by a gamma-aminobutyric acid-A-receptor antagonistic effect J Pharmacol Exp Ther, 301: 168-173
  • Kristufek D, Rudorfer W, Pifl C, Huck S (2002) Organic cation transporter mRNA and function in the rat superior cervical ganglion J Physiol, 543: 117-134
  • Lugowska A., Berger J., Tylki-Szymanska A., Czartoryska B., Löschl B., Molzer B (2002) High prevalence of I179S mutation in patients with late-onset metachromatic leukodystrophy Clin Genet, 61: 389-390
  • Tylki-Szymanska A., Lugowska A., Chmielik J., Kotowicz J., Winiecka A., Zobel M., Berger J., Molzer B (2002) Investigation into micro-organic brain damage (MOBD) in heterozygotes of metachromatic leukodystrophy Am J Med Genet, 110: 315-319
  • Weinhofer I., Forss-Petter S., Zigman M., Berger J (2002) Cholesterol regulates ABCD2 expression: Implication for therapy of X-linked adrenoleukodystrophy Hum Mol Genet, 11: 2701-2708

2001 Original Articles

  • Scholze P, Sitte H, Singer E (2001) Substantial loss of substrate by diffusion during uptake in HEK-293 cells expressing neurotransmitter transporters Neurosci Lett, 309 (3): 173-176
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  • Schmid J, Scholze P, Kudlacek O, Freissmuth M, Singer E, Sitte H (2001) Oligomerization of the Human Serotonin Transporter and of the Rat GABA Transporter 1 Visualized by Fluorescence Resonance Energy Transfer Microscopy in Living Cells J Biol Chem, 276 (6): 3805-3810
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  • Klausberger T, Sarto I, Ehya N, Fuchs K, Furtmüller R, Mayer B, Huck S, Sieghart W (2001) Alternate use of distinct intersubunit contacts controls GABA-A receptor assembly and stoichiometry J Neurosci, 21: 9124-9133
  • Sitte HH, Hiptmair B, Zwach J, Pifl C, Singer EA, Scholze P (2001) Quantitative analysis of inward and outward transport rates in cells stably expressing the cloned human serotonin transporter: inconsistencies with the hypothesis of facilitated exchange diffusion Mol Pharmacol, 59: 1129-1137
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  • Albet S., Bentejac M., Savary S., Gondcaille C., Netik A., Berger J., Szpirer C., Pineau T., Toiffer-Charlier N., Bugaut M (2001) Rat adrenoleukodystrophy-related (ALDR) gene: full-length cDNA sequence and new insight in expression Biochim Biophys Acta, 1517: 257-269
  • Dvoráková L., Štorkánová G., Unterrainer G. , Hujová J., Kmoch S., Zeman J., Høebíèek M., Berger J (2001) Eight novel ABCD1 gene mutations and three polymorphisms in patients with X-linked adrenoleukodystrophy: The first polymorphism causing an amino acid exchange Hum Mut, 18: 52-60
  • Fourcade S., Savary S, Albet S, Gauthé D., Gondcaille C., Pineau T., Bellenger J., Bentejac M., Holzinger A., Berger J., Bugaut M (2001) Fibrate induction of the Adrenoleukodystrophy-Related gene (ABCD2): promoter analysis and role of the peroxisome proliferator-activated receptor PPARa Eur J Biochem, 268: 3490-3500

2000 Original Articles

  • Sitte HH, Scholze P, Schloss P, Pifl C, Singer EA (2000) Characterization of carrier-mediated efflux in human embryonic kidney 293 cells stably expressing the rat serotonin transporter: a superfusion study J Neurochem, 74: 1317-1324
  • Scholze P, Zwach J, Kattinger A, Pifl V, Singer EA, Sitte HH (2000) Transporter-mediated release: a superfusion study on human embryonic kidney cells stably expressing the human serotonin transporter J Pharmacol Exp Ther, 293: 870-878p
  • Nöhammer C., El Shabrawi Y., Schauer S., Hiden M., Berger J., Forss-Petter S., Winter E., Eferl R., Zechner R., Höfler G. (2000) cDNA cloning and analysis of issue-specific expression of mouse peroxisomal acyl-CoA oxidase Eur J Biochem, 267: 1254-1260
  • Lugowska A., Czartoryska B., Tylki-Szymanska A., Bisko M., Zimowski JG., Berger J., Molzer B (2000) Prevalence of arylsulfatase A pseudodeficiency allele in metachromatic leukodystrophy patients from Poland Eur Neurol, 44: 104-107
  • Unterrainer G., Molzer B., Forss-Petter S., Berger J (2000) Coexpression of mutated adrenoleukodystrophy protein reduces wild-type protein function: Implication for gene therapy in X-linked adrenoleukodystrophy Hum Mol Genet, 9: 2609-2616
  • Netik A., Hobel A., Rauschka H., Molzer B., Forss-Petter S., Berger J (2000) Rolipram does not normalize very long chain fatty acid levels in adrenoleukodystrophy protein-deficient fibroblasts and mice J Inhereted Metab Dis, 23: 615-624
  • Berger J., Korosec T., Unterrainer G., Molzer B (2000) A de novo adrenoleukodystrophy gene (ABCD1) mutation S636I without detectable ABCD1 protein and a R104C mutation with normal amount of protein from a Austrian patient collective Hum Mut, 16: 534

1999 Original Articles

  • Thomet U, Baur R, Scholze P, Sieghart W, Sigel E (1999) Dual mode of stimulation by the beta-carboline ZK91085 of recombinant GABA-A receptor currents: molecular determinants affecting its action Br J Pharmacol, 127: 1231-1239
  • Ebert V, Scholze P, Fuchs K, Sieghart W (1999) Identification of subunits mediating clustering of GABA-A receptors by rapsyn Neurochem Internat, 34: 453-463
  • Berger J., Gmach M., Mayr H., Molzer B., Bernheimer H (1999) Coincidence of two novel arylsulfatase A alleles and mutation 459+1G>A within a family with metachromatic leukodystrophy: molecular basis of phenotypic heterogeneity Hum Mut, 13: 61-68
  • Netik A., Forss-Petter S., Holzinger A., Molzer B., Unterrainer G., Berger J (1999) Adrenoleukodystrophy-related protein can functionally compensate for adrenoleukodystrophy protein deficiency (X-ALD): implications for therapy Hum Mol Genet, 8: 907-913
  • Holzinger A., Mayerhofer P., Berger J., Lichtner P., Krammerer S., Roscher A. (1999) Full length cDNA cloning, promoter sequence and genomic organisatin of the human adrenoleukodystrophy related (ALDR) gene functionally redundant to the gene responsible for X-linked adrenoleukodystrop Biochem Bioph Res Comm, 258: 436-442
  • Berger J., Albet S., Bentejac M., Netik A., Holzinger A., Roscher A., Bugaut M., Forss-Petter S (1999) The four murine peroxisomal ABC-transporter genes differ in constitutive, inducible and developmental expression Eur J Biochem, 265: 719-727

1998 Original Articles

  • Sitte HH, Huck S, Reither H, Boehm S, Singer EA, Pifl C (1998) Carrier-mediated release, transport rates and charge transfer induced by amphetamine, tyramine and dopamine in mammalian cells transfected with the human dopamine transporter J Neurochem, 71: 1289-1297
  • Berger J., Truppe C., Neumann H., Forss-Petter S (1998) A novel relative of the very long-chain acyl-CoA synthetase and fatty acid transporter protein genes with a distinct expression pattern Biochem Bioph Res Comm, 247: 255-260
  • Berger J., Truppe C., Neumann H., Forss-Petter S. (1998) cDNA cloning and mRNA distribution of a mouse very long-chain acyl-CoA synthetase FEBS Letters, 425: 305-309
  • Santo-Matsumura K.C., Berger J., Hainfellner J.A., Mazal P., Budka H (1998) Development of HIV encephalitis in AIDS and TNF-a regulatory elements J Neuroimmunol, 91: 89-92
  • Lametschwandtner G., Brocard C., Fransen M., Veldhoven P.V., Berger J., Hartig A (1998) The difference in recognition of terminal tripeptides as peroxisomal targeting signal 1 between yeast and human is due to different affinities of their receptor Pex5p to the cognate signal and to resi J Biol Chem, 273: 33635-33643
  • Bauer, J., Bradl, M., Hickey, W.F., Forss-Petter, S., Breitschopf, H., Linington, C., Wekerle, H., and Lassmann, H (1998) T cell apoptosis in inflammatory brain lesions. Destruction of T cells does not depend on antigen recognition Am.J.Pathol., 153: 715-724

1997 Original Articles

  • Forss-Petter S, Werner H, Berger J, Lassmann H, Molzer B, Schwab MH, Bernheimer H, Zimmermann F, Nave KA (1997) Targeted inactivation of the X-linked adrenoleucodystrophy gene in mice. J Neurosci Res, 50: 829-843
  • Weissenböck H., Hainfellner J.A., Berger J., Kasper I., Budka H (1997) Naturally occuring herpes simplex encephalitis in a domestic rabbit (Oryctolagus cuniculus) Vet Pathol, 34: 44-47
  • McGuinness M.C., Powers J.M., Bias W.B., Schmeckpeper B.J., Segal A.H., Gowda V.C., Wesselingh S.L., Berger J., Griffin D.E., Smith K.D. (1997) Human leukocyte antigens and cytokine expression in cerebral inflammmatory demyelinative lesions of X-linked adrenoleukodystrophy and multiple sclerosis J Neuroimmunol, 75: 174-182
  • Berger J., Löschl B., Bernheimer H., Lugowska A., Tylki-Szymanska A., Gieselmann V., Molzer B (1997) Occurrence, distribution, and phenotype of arylsulfatase A mutations in patients with metachromatic leukodystrophy Am J Med Genet, 69: 335-340
  • Lugowska A., Tylki-Szymanska A., Berger J., Molzer B (1997) Elevated sulfatide excretion in compound heterozygotes of metachromatic leukodystrophy and ASA-pseudodeficiency alleles Clin Biochem, 30: 325-331
  • Holzinger A., Kammerer S., Berger J., Roscher A.A (1997) cDNA cloning and mRNA expression of the human adrenoleukodystrophy related protein (ALDR), a peroxisomal ABC-transporter Biochem Bioph Res Comm, 239: 261-264

1997 Reviews

  • Berger J., Bernheimer H., Forss-Petter S (1997) False PCR-based diagnosis: is it avoidable? Am J Med Genet, 72: 241

1996 Original Articles

  • Tylki-Szymanska A., Berger J., Löschl B., Lugowska A., Molzer B (1996) Late juvenile metachromatic leukodystrophy (MLD) in three patients with similar clinical course and identical mutation on one allele Clin Genet, 50: 287-292
  • Madsen K.M., Hasholt L., Berger J., Sorenson S.A. (1996) SSCP analysis of paraffin embeddes tissues in a family with an atypical form of Fabry disease J Clin Pathol; Mol Pathol, 49: 310-312
  • Berger J., Gmach M., Faé I., Molzer B., Bernheimer H (1996) A new polymorphism of arylsulfatase A within the coding region Hum Genet, 98: 348-350

1995 Original Articles

  • Berger J., Bernheimer H., Fáe I., Braun A., Roscher A., Molzer B., Fischer G. (1995) Association of X-linced adrenoleukodystrophy with HLA DRB1 alleles Biochem Bioph Res Comm, 216: 447-451

1994 Original Articles

  • Berger J., Molzer B., Fáe I., Bernheimer H. (1994) X-linked adrenoleukodystrophy (ALD): A novel mutation of the ALD gene in 6 members of a family presenting with 5 different phenotypes Biochem Bioph Res Comm, 205: 1638-1643

1993 Original Articles

  • Berger J., Molzer B., Gieselmann V., Bernheimer H. (1993) Simultaneous detection of the two most frequent metachromatic leukodystrophy mutations Hum Genet, 92: 421-423

1992 Original Articles

  • Weichselbraun I., Berger J., Dobrovnik M., Bogerd H., Grassmann R., Greene W.C., Hauber J., Böhnlein E (1992) Dominant-Negative Mutants Are Clustered in a Domain of the Human T-Cell Leukemia Virus Type I Rex Protein: Implications for trans Dominace J Virol, 66: 4540-4545

1991 Original Articles

  • Berger J., Aepinus C., Dobrovnik M., Fleckenstein B., Hauber J., Böhnlein E. (1991) Mutational Analysis of Functional Domains in the HIV-1 Rev trans-Regulatory Protein Virology, 183: 630-635
  • Böhnlein E., Berger J., Hauber J. (1991) Functional Mapping of the Human Immunodeficiency Virus Type 1 Rev RNA Binding Domain: New Insights into the Domain Structure of Rev and Rex J Virol, 65: 7051-7055

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