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Detail

Johannes Berger
Univ.Prof. Dr. Johannes BergerHead of Division of Pathobiology of the Nervous System

Center for Brain Research (Division of Pathobiology of the Nervous System)
Position: Professor

T +43 1 40160 34300
johannes.berger@meduniwien.ac.at

Further Information

Keywords

Adrenoleukodystrophy; Alzheimer Disease; Peroxisomal Disorders; Peroxisomes; Plasmalogens

Research interests

My main research focus is to understand the importance of different peroxisomal metabolic pathways for the proper function of the brain. The research is designed to elucidate molecular mechanisms underlying the different phenotypes of X-linked adrenoleukodystrophy and to develop novel therapeutic approaches. The ether phospholipid biosynthesis is disturbed in a group of inherited peroxisomal disorders but increasing evidence emerges suggesting a role of ether phospholipids in the pathogenesis of late onset neurodegenerative diseases such as Alzheimer’s disease. Thus, the characterization of molecular consequence of ether phospholipid deficiency for the nervous system is another focus of our laboratory.

Techniques, methods & infrastructure

Mouse models for X-linked adrenoleukodystrophy, ether phospholipid deficiency or Alzheimer’s disease are used for in vivo analysis and primary cells from human patients (e.g. monocytes, lymphoblast or fibroblasts) for in vitro analysis of the role of peroxisomes in proper cellular functioning. A broad spectrum of biochemical, cell biological, and imaging techniques are used.

Grants

  • Übernahme der Reise- u. Übernachtungskosten für AMN Patienten aus Deutschland zur Teilnahme an einer klinischen Studie (2014)
    Source of Funding: ELA Deutschland,
    Principal Investigator
  • Characterization of immune cells in X-linked adrenoleukodystrophy (2013)
    Source of Funding: FWF (Austrian Science Fund), Stand-Alone Projects
    Principal Investigator
  • Neurotransmitter Alterations in Ether Lipid Deficiency (2012)
    Source of Funding: FWF (Austrian Science Fund), Stand-Alone Projects
    Principal Investigator
  • LeukoTreat - Therapeutic challenge in Leukodystrophies: Translation and ethical research towards clinical trials (project partner) (2010)
    Source of Funding: EU, FP7-HEALTH-2009
    Principal Investigator

Selected publications

  1. 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
  2. Weber F, Wiesinger Ch, Forss-Petter S, Regelsberger G, Einwich A, Weber W, Kohler 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 | Article (PDF) |
  3. 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
  4. 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.
  5. Kou J, Kovacs GG, Hoftberger R, Kulik W, Brodde A, Forss-Petter S, Honigschnabl 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 |