Department: Division of Pathobiology of the Nervous System
Head: Weinhofer-Molisch, Isabelle, Ap. Prof. Mag. Dr.
Research Area
Neuroinflammation, X-linked Adrenoleukodystrophy
Description
I am fascinated by the complexity of X-linked adrenoleukodystrophy (X-ALD). In X-ALD, the very long-chain fatty acid metabolism is disturbed resulting in a clinically heterogeneous neurometabolic disorder. As it is still unclear why more than half of all patients develop the severe neuroinflammatory form that results in death if not recognized early enough, my research is designed to elucidate molecular mechanisms underlying the different phenotypes and to develop novel therapeutic approaches. By clarifying the role of macrophages, we made an important contribution to understand how life-saving hematopoietic stem cell transplantation is able to halt the inflammation in X-ALD. A new but very urgent research focus is to establish an easy accessible early blood biomarker that would indicate the onset of cerebral inflammation in X-ALD patients.
Selected Publications of Principle Investigator
- 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
- 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): 305
- 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
- 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
- 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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