Isabelle Weinhofer

Abstract:

I am fascinated by 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 neurodegenerative, neuroinflammatory and demyelinating disease. My current research is designed to elucidate molecular mechanisms underlying the different phenotypes of X-ALD and to develop novel therapeutic approaches. A novel research focus is the Epstein-Barr virus and its role in modulating peroxisomal metabolism which could be critical in the initiation of the neuroinflammatory form of X-ALD.

Techniques:

·         Isolation, culture and analysis of human primary immune cells

·         Gene expression studies

·         Immunostainings

·         Epstein-Barr virus isolation and infection of primary cells

·         Exosome isolation and characterization

Selected Publications:

1. 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)
2. 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
3. Zierfuss B, Weinhofer I, Buda A, Popitsch N, Hess L, Moos V, Hametner S, Kemp S, Köhler W, Forss‐Petter S, Seiser C, Berger J (2020) Targeting foam cell formation in inflammatory brain diseases by the histone modifier MS‐275 Ann Clin Transl Neurol,
4. Zierfuss B, Weinhofer I, Kühl J, Köhler W, Bley A, Zauner K, Binder J, Martinović K, Seiser C, Hertzberg C, Kemp S, Egger G, Leitner G, Bauer J, Wiesinger C, Kunze M, Forss-Petter S, Berger J (2020) Vorinostat in the acute neuroinflammatory form of X-linked adrenoleukodystrophy Ann Clin Transl Neurol, 7 (5): 639-652
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