Mag. Dr. Isabelle Weinhofer-Molisch

Department(s): Department of Pathobiology of the Nervous System (Center for Brain Research)
Position: Research Assistant
Location: Spitalgasse 4
Telephone: 40160-34091
E-Mail:

Research Area:
Peroxisomes in Brain Disorders

Research Interests:
Peroxisomes, Macrophages, Herpesviridae, Adrenoleukodystrophy

Grant(s):

• Die Rolle von Peroxisomen im Hormonstoffwechsel (Responsible)
• Übernahme der Reise- u. Übernachtungskosten für AMN Patienten aus Deutschland zur Teilnahme an einer klinischen Studie

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, 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) |
2. Wolff P, Weinhofer I, Seguin J, Roszak P, Beisel C, Donoghue MT, Spillane C, Nordborg M, Rehmsmeier M, Köhler C. (2011) High-resolution analysis of parent-of-origin allelic expression in the Arabidopsis Endosperm PLoS Genet, 7(6): e1002126.
3. Weinhofer I, Hehenberger E, Roszak P, Hennig L, Köhler C. (2010) H3K27me3 profiling of the endosperm implies exclusion of polycomb group protein targeting by DNA methylation PLoS Genet, 6(10): e1001152
4. Weinhofer I., Kunze M., Rampler H., Bookout A.L., Forss-Petter S., Berger J (2005) LXRalpha interferes with SREBP1c-mediated Abcd2 expression: novel cross-talk in gene regulation J Biol Chem, 280: 41243-41251
5. Weinhofer I., Forss-Petter S., Zigman M., Berger J (2002) Cholesterol regulates ABCD2 expression: Implication for therapy of X-linked adrenoleukodystrophy Hum Mol Genet, 11: 2701-2708

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