Department: Division of Pathobiology of the Nervous System
Head: Berger, Johannes, Univ.Prof. Dr.
Research Area
Peroxisomes in Brain Disorders
Description
Our main research focus is to understand the importance and functioning of different peroxisomal metabolic pathways such as the degradation of very long-chain fatty acids or the biosynthesis of ether phospholipids for the proper function of the brain.
The very long-chain fatty acid metabolism is disturbed in X-linked adrenoleukodystrophy, a clinically heterogonous neurodegenerative, neuroinflammatory and demyelinating disease. Our 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.
Members
Selected Publications of Principle Investigator
- Weinhofer I, Rommer P, Zierfuss B, Altmann P, Foiani M, Heslegrave A, Zetterberg H, Gleiss A, Musolino P, Gong Y, Forss-Petter S, Berger T, Eichler F, Aubourg P, Köhler W, Berger J (2021) Neurofilament light chain as a potential biomarker for monitoring neurodegeneration in X-linked adrenoleukodystrophy Nat Commun, 12 (1): 1816
- 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
- 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
| Article (PDF) | - 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.
- 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
| Article (PDF) |