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Medical NeuroScience Cluster Publications by Berger, Johannes, Univ.Prof. Dr.

2024 Original Articles

  • 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

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
  • 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
  • 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
  • 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

  • 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
  • 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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  • 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

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
  • 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
  • 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)

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)
  • 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,
  • 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
  • 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
  • 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

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

  • 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
  • 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

2018 Original Articles

  • 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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2017 Original Articles

  • 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 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
  • 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
  • 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
  • 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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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

  • 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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  • 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 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

  • Schmied M, Zehetmayer S, Reindl M, Ehling R, Bajer-Kornek B, Leutmezer F, Zebenholzer K, Hotzy C, Lichtner P, Meitinger T, Wichmann H, Illig T, Gieger C, Huber K, Khalil M, Fuchs S, Schmidt H, Auff E, Kristoferitsch W, Fazekas F, Berger T, Vass K, Zimprich A (2012) Replication study of multiple sclerosis (MS) susceptibility alleles and correlation of DNA-variants with disease features in a cohort of Austrian MS patients Neurogenetics, 13 (2): 181-187
  • 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
  • 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

  • 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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2010 Original Articles

  • 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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2010 Reviews

  • 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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2008 Original Articles

  • 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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2007 Original Articles

  • 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

2005 Original Articles

  • 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
  • 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

  • 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

  • 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

  • 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

  • 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

  • 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

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