Full Info Person

Research Area

Immunity, Innate, Peroxisomes in Brain Disorders

Research Interests

Adrenoleukodystrophy, Neuroinflammation, Peroxisomes

Interest Group(s)

• X-linked Adrenoleukodystrophy

Grant(s)

• Die Rolle von Peroxisomen im Hormonstoffwechsel (Head)
• Liquid biopsy for peripheral disease monitoring in X-ALD (Head)
• Liquid biopsy for peripheral disease monitoring in X-ALD (Head)

Abstract

I am fascinated by the complexity of the peroxisomal disorder X-linked adrenoleukodystrophy (X-ALD). In X-ALD, the very long-chain fatty acid metabolism is disturbed resulting in a clinically heterogeneous neurometabolic disease. 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 focused on innate immune cells and designed to elucidate molecular mechanisms underlying the different phenotypes and to develop novel therapeutic approaches. 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.

Techniques

• Isolation, culture and anaylsis of human primary immune cells
• iPSC culture and differentiation to different neural cell types
• gene expression studies, FACS, live imaging and other biochemical, cell biological and imaging techniques

Selected publications

1. 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
2. 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)
3. 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)
4. 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
5. 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) |

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