Johannes Berger

Summary

Affiliation: Medical University of Vienna
Country: Austria

Publications

  1. pmc Peroxisomal alterations in Alzheimer's disease
    Jianqiu Kou
    Center for Brain Research, Medical University of Vienna, Vienna, Austria
    Acta Neuropathol 122:271-83. 2011
  2. doi request reprint Pathophysiology of X-linked adrenoleukodystrophy
    J Berger
    Pathobiology of the Nervous System, Center for Brain Research, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria Electronic address
    Biochimie 98:135-42. 2014
  3. ncbi request reprint X-linked adrenoleukodystrophy: clinical, biochemical and pathogenetic aspects
    Johannes Berger
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria
    Biochim Biophys Acta 1763:1721-32. 2006
  4. pmc Current and future pharmacological treatment strategies in X-linked adrenoleukodystrophy
    Johannes Berger
    Center for Brain Research, Medical University of Vienna, Vienna, Austria
    Brain Pathol 20:845-56. 2010
  5. doi request reprint Distinct modulatory roles for thyroid hormone receptors TRalpha and TRbeta in SREBP1-activated ABCD2 expression
    Isabelle Weinhofer
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria
    Eur J Cell Biol 87:933-45. 2008
  6. ncbi request reprint Liver X receptor alpha interferes with SREBP1c-mediated Abcd2 expression. Novel cross-talk in gene regulation
    Isabelle Weinhofer
    Center for Brain Research, Medical University Vienna, A 1090 Vienna, Austria
    J Biol Chem 280:41243-51. 2005
  7. ncbi request reprint X-linked adrenoleukodystrophy mice demonstrate abnormalities in cholesterol metabolism
    Isabelle Weinhofer
    Center for Brain Research, Medical University Vienna, Austria
    FEBS Lett 579:5512-6. 2005
  8. pmc Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction
    Christoph Wiesinger
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090 Vienna, Austria
    J Biol Chem 288:19269-79. 2013
  9. doi request reprint Peroxisomal localization of the proopiomelanocortin-derived peptides beta-lipotropin and beta-endorphin
    Romana Hoftberger
    Institute of Neurology, Center for Brain Research, Medical University of Vienna, and Department of Neurology, SMZ Ost Danube Hospital, AKH 4J, Wahringer Gurtel 18 20, P O Box 48, A 1097 Vienna, Austria
    Endocrinology 151:4801-10. 2010
  10. ncbi request reprint Distribution and cellular localization of adrenoleukodystrophy protein in human tissues: implications for X-linked adrenoleukodystrophy
    Romana Hoftberger
    Institute of Neurology, Medical University of Vienna, AKH 4J, Wahringer Gurtel 18 20, POB 48, A 1097 Vienna, Austria
    Neurobiol Dis 28:165-74. 2007

Collaborators

Detail Information

Publications32

  1. pmc Peroxisomal alterations in Alzheimer's disease
    Jianqiu Kou
    Center for Brain Research, Medical University of Vienna, Vienna, Austria
    Acta Neuropathol 122:271-83. 2011
    ..These results indicate substantial peroxisome-related alterations in AD, which may contribute to the progression of AD pathology...
  2. doi request reprint Pathophysiology of X-linked adrenoleukodystrophy
    J Berger
    Pathobiology of the Nervous System, Center for Brain Research, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria Electronic address
    Biochimie 98:135-42. 2014
    ..An improved understanding of the molecular mechanisms involved in these events is required for the development of urgently needed therapeutics. ..
  3. ncbi request reprint X-linked adrenoleukodystrophy: clinical, biochemical and pathogenetic aspects
    Johannes Berger
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria
    Biochim Biophys Acta 1763:1721-32. 2006
    ..Urgently needed novel therapeutic strategies are under consideration ranging from dietary approaches to gene therapy...
  4. pmc Current and future pharmacological treatment strategies in X-linked adrenoleukodystrophy
    Johannes Berger
    Center for Brain Research, Medical University of Vienna, Vienna, Austria
    Brain Pathol 20:845-56. 2010
    ....
  5. doi request reprint Distinct modulatory roles for thyroid hormone receptors TRalpha and TRbeta in SREBP1-activated ABCD2 expression
    Isabelle Weinhofer
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria
    Eur J Cell Biol 87:933-45. 2008
    ..These findings show that TRalpha and TRbeta differentially modulate SREBP1-activated ABCD2 expression at overlapping SRE/DR-4 elements, suggesting a novel mode of cross-talk between TR and SREBP in gene regulation...
  6. ncbi request reprint Liver X receptor alpha interferes with SREBP1c-mediated Abcd2 expression. Novel cross-talk in gene regulation
    Isabelle Weinhofer
    Center for Brain Research, Medical University Vienna, A 1090 Vienna, Austria
    J Biol Chem 280:41243-51. 2005
    ..Together, we identify LXRalpha as negative modulator of Abcd2, acting through a novel regulatory mechanism involving overlapping SREBP and LXRalpha binding sites...
  7. ncbi request reprint X-linked adrenoleukodystrophy mice demonstrate abnormalities in cholesterol metabolism
    Isabelle Weinhofer
    Center for Brain Research, Medical University Vienna, Austria
    FEBS Lett 579:5512-6. 2005
    ..In murine X-ALD fibroblasts, cholesterol-lowering did not normalize VLCFA. Thus, ALDP-deficiency and VLCFA are linked to cholesterol but species differences complicate evaluating cholesterol-lowering drugs in X-ALD mice...
  8. pmc Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction
    Christoph Wiesinger
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090 Vienna, Austria
    J Biol Chem 288:19269-79. 2013
    ..In conclusion, our findings in human cells suggest that, in contrast to yeast cells, very long-chain acyl-CoA esters are transported into peroxisomes by ABCD1 independently of additional synthetase activity. ..
  9. doi request reprint Peroxisomal localization of the proopiomelanocortin-derived peptides beta-lipotropin and beta-endorphin
    Romana Hoftberger
    Institute of Neurology, Center for Brain Research, Medical University of Vienna, and Department of Neurology, SMZ Ost Danube Hospital, AKH 4J, Wahringer Gurtel 18 20, P O Box 48, A 1097 Vienna, Austria
    Endocrinology 151:4801-10. 2010
    ..It remains to be elucidated whether and how the peroxisomal localization of POMC-derived hormones has a role in the endocrine dysfunction of peroxisomal disease...
  10. ncbi request reprint Distribution and cellular localization of adrenoleukodystrophy protein in human tissues: implications for X-linked adrenoleukodystrophy
    Romana Hoftberger
    Institute of Neurology, Medical University of Vienna, AKH 4J, Wahringer Gurtel 18 20, POB 48, A 1097 Vienna, Austria
    Neurobiol Dis 28:165-74. 2007
    ..This might indicate a functional link between ALDP and proopiomelanocortin-derived peptide hormones...
  11. ncbi request reprint Lack of adrenoleukodystrophy protein enhances oligodendrocyte disturbance and microglia activation in mice with combined Abcd1/Mag deficiency
    Martina Dumser
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090, Vienna, Austria
    Acta Neuropathol 114:573-86. 2007
    ..Furthermore, the age at which this occurs precedes by far the onset of axonal degeneration in Abcd1-deficient mice, implying that oligodendrocyte/myelin disturbances may precede axonopathy in X-ALD...
  12. ncbi request reprint A novel mammalian bubblegum-related acyl-CoA synthetase restricted to testes and possibly involved in spermatogenesis
    Peter Fraisl
    Center for Brain Research, Division of Neuroimmunology, Medical University of Vienna, Spitalgasse 4, A 1090 Vienna, Austria
    Arch Biochem Biophys 451:23-33. 2006
    ..Thus, the BGR gene expands the bubblegum ACS family with a testes-specific, developmentally regulated member that may play a role in spermatogenesis...
  13. ncbi request reprint Peroxisomal cholesterol biosynthesis and Smith-Lemli-Opitz syndrome
    Isabelle Weinhofer
    Center for Brain Research, Medical University of Vienna, Vienna, Austria
    Biochem Biophys Res Commun 345:205-9. 2006
    ..Our results implicate peroxisomes in cholesterol biosynthesis but provide no link to phenotypic variation in SLOS...
  14. pmc Structural requirements for interaction of peroxisomal targeting signal 2 and its receptor PEX7
    Markus Kunze
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090 Vienna, Austria
    J Biol Chem 286:45048-62. 2011
    ..This additional information on PTS2 signals was used to generate a PTS2 prediction algorithm that enabled us to identify novel PTS2 signals within human proteins and to describe KChIP4 as a novel peroxisomal protein...
  15. ncbi request reprint Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency
    Iris Oezen
    Center for Brain Research, Medical University Vienna, Vienna, Austria
    Hum Mol Genet 14:1127-37. 2005
    ..Thus, we conclude that the accumulation of VLCFA per se does not cause mitochondrial abnormalities and vice versa-mitochondrial abnormalities are not responsible for the accumulation of VLCFA in X-ALD mice...
  16. ncbi request reprint Lessons from knockout mice II: Mouse models for peroxisomal disorders with single protein deficiency
    Johannes Berger
    Brain Research Institute, University of Vienna, Spitalgasse 4, 1090 Vienna, Austria
    Adv Exp Med Biol 544:123-34. 2003
  17. doi request reprint X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism is severely impaired in monocytes but not in lymphocytes
    Franziska D Weber
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, Vienna A 1090, Austria
    Hum Mol Genet 23:2542-50. 2014
    ..Additionally, these findings support the concept that ABCD2 is a target for pharmacological induction as an alternative therapeutic strategy. ..
  18. doi request reprint Involvement of human peroxisomes in biosynthesis and signaling of steroid and Peptide hormones
    Isabelle Weinhofer
    Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090, Vienna, Austria
    Subcell Biochem 69:101-10. 2013
    ..With this chapter, we will review several crucial aspects concerning peroxisomes and hormone metabolism. ..
  19. ncbi request reprint Evaluation of the therapeutic potential of PPARalpha agonists for X-linked adrenoleukodystrophy
    Heidelinde Rampler
    Brain Research Institute, University of Vienna, Vienna, Austria
    Mol Genet Metab 80:398-407. 2003
    ..Our results suggest that the PPARalpha agonist-mediated induction of Abcd2 expression seems to be indirect and possibly mediated by SREBP2...
  20. ncbi request reprint Proteome profiling in the rat Harderian gland
    Jae Won Yang
    Department of Pediatrics, Medical University of Vienna, Vienna, Austria
    J Proteome Res 5:1751-62. 2006
    ....
  21. ncbi request reprint Expression of hypoxia-related tissue factors correlates with diminished survival of adjuvantly treated patients with chromosome 1p aberrant oligodendroglial neoplasms and therapeutic implications
    Peter Birner
    Institute of Neurology, Medical University Vienna, Vienna, Austria
    Clin Cancer Res 10:6567-71. 2004
    ..In this study, we evaluated expression of hypoxia-related factors and its influence on survival in oligodendroglial brain tumors with chromosome 1p aberrations...
  22. pmc Murine bubblegum orthologue is a microsomal very long-chain acyl-CoA synthetase
    Peter Fraisl
    Division of Neuroimmunology, Brain Research Institute, Vienna University Medical School, Spitalgasse 4, 1090 Vienna, Austria
    Biochem J 377:85-93. 2004
    ..Although mouse Bgm is capable of activating VLCFA, we conclude that a direct interaction between the mouse Bgm and the Aldp seems unlikely...
  23. ncbi request reprint Thyroid hormone induction of the adrenoleukodystrophy-related gene (ABCD2)
    Stephane Fourcade
    Laboratoire de Biologie Moleculaire et Cellulaire, Faculte des Sciences Gabriel, Universite de Bourgogne, 6 Boulevard Gabriel, 21000 Dijon, France
    Mol Pharmacol 63:1296-303. 2003
    ..Finally, ABCD3 (PMP70), a close homolog of ABCD2, was also induced by T3 in the liver of control rats, but not that of TRbeta-/- mice, and in CG4 oligodendrocytes...
  24. ncbi request reprint Pharmacological induction of redundant genes for a therapy of X-ALD: phenylbutyrate and other compounds
    Maurice Bugaut
    Laboratory of Molecular and Cellular Biology, Faculty of Sciences Gabriel, University of Burgundy 21000 Dijon, France
    Adv Exp Med Biol 544:281-91. 2003
  25. ncbi request reprint Cholesterol regulates ABCD2 gene expression: implications for X-linked adrenoleukodstrophy
    Isabelle Weinhofer
    Brain Research Institute, University of Vienna, Spitalgasse 4 A 1090 Vienna, Austria
    Adv Exp Med Biol 544:331-2. 2003
  26. ncbi request reprint Mouse very long-chain Acyl-CoA synthetase 3/fatty acid transport protein 3 catalyzes fatty acid activation but not fatty acid transport in MA-10 cells
    Zhengtong Pei
    Kennedy Krieger Research Institute and Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Biol Chem 279:54454-62. 2004
    ..These studies cast doubt on the designation of ACSVL3 as a fatty acid transport protein...
  27. ncbi request reprint Cholesterol regulates ABCD2 expression: implications for the therapy of X-linked adrenoleukodystrophy
    Isabelle Weinhofer
    Brain Research Institute, University of Vienna, Austria
    Hum Mol Genet 11:2701-8. 2002
    ..Thus, lowering cholesterol leads to SREBP maturation, increased ABCD2 expression and reduced VLCFA accumulation...
  28. ncbi request reprint Evidence against the adrenoleukodystrophy-related gene acting as a modifier of X-adrenoleukodystrophy
    Andreas Holzinger
    Dr von Hauner Children s Hospital, Ludwig Maximilians University Munich, Lindwurmstrasse 4, 80447 Munich, Germany
    Adv Exp Med Biol 544:95-6. 2003
  29. ncbi request reprint Proteomics characterization of mouse kidney peroxisomes by tandem mass spectrometry and protein correlation profiling
    Sebastian Wiese
    Medizinisches Proteom Center, Ruhr Universitaet Bochum, Universitaetsstrasse 150, 44780 Bochum, Germany
    Mol Cell Proteomics 6:2045-57. 2007
    ..As a result of this joint effort, we believe to have compiled the so far most comprehensive protein catalogue of mammalian peroxisomes...
  30. ncbi request reprint A new paraclinical CSF marker for hypoxia-like tissue damage in multiple sclerosis lesions
    Hans Lassmann
    Division of Neuroimmunology, Brain Research Institute, University of Vienna, Austria
    Brain 126:1347-57. 2003
    ..The respective epitope is liberated into the CSF and, thus, may become a useful diagnostic tool to identify clinically a defined multiple sclerosis subtype...
  31. ncbi request reprint Brain neutral lipids mass is increased in alpha-synuclein gene-ablated mice
    Gwendolyn Barcelo-Coblijn
    Department of Pharmacology, Physiology, and Therapeutics, University of North Dakota, Grand Forks, North Dakota 58202 9037, USA
    J Neurochem 101:132-41. 2007
    ..These data extend our previous results in astrocytes and kinetic studies in vivo demonstrating a role for Snca in brain lipid metabolism and demonstrate a clear impact on brain neutral lipid metabolism...
  32. pmc Hidden localization motifs: naturally occurring peroxisomal targeting signals in non-peroxisomal proteins
    Georg Neuberger
    Research Institute of Molecular Pathology IMP, Dr Bohr Gasse 7, A 1030 Vienna, Austria
    Genome Biol 5:R97. 2004
    ....