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Imagine a big city without a car driving and no mail being delivered. The resulting chaos is similar to what happens to a cell when its molecular transport systems are impaired. Our goal is to understand the molecular principles underlying cargo recognition by transport complexes, complex assembly and activation, and eventually complex disassembly after the transport. Our research tools are X-ray crystallography, quantitative biophysical approaches, biochemistry, and in vivo studies.
As a first model, we analyzed the directional transport of ASH1 mRNA in S. cerevisiae. Since then, we expanded our research to neuronal mRNA-protein complexes and their specificity in binding. Two of these neuronal projects involve RNA-binding proteins that have been associated with neuronal disorders:
PURA Syndrome: PURA is an ubiquitously expressed protein with enrichment in the brain. Spontaneous mutations in its gene result in the rare neurodevelopmental disorder PURA Syndrome. We are closely interacting with the patient-centered PURA Syndrome Foundation to understand which molecular and cellular pathways are affected in this disorder and result in the patient's symptoms. Towards this goal, we combine our classical structural and biochemical approaches with cell-culture studies, including iPSC-based analyses, and various omics approaches.
Poly-glutamine diseases: These diseases are caused by pathological genomic expansions of disease-related genes that are translated into polyQ stretches. Proteins with such expanded polyQ stretches tend to form neurotoxic aggregates. Together with our collaboration partners, we are characterizing the therapeutic potential of a novel drug target towards a preventive treatment of affected patients in early stages of their disease.
Imagine a big city without a car driving and no mail being delivered. The resulting chaos is similar to what happens to a cell when its molecular transport systems are impaired. Our goal is to understand the molecular principles underlying cargo recognition by transport complexes, complex assembly and activation, and eventually complex disassembly after the transport. Our research tools are X-ray crystallography, quantitative biophysical approaches, biochemistry, and in vivo studies.
As a first model, we analyzed the directional transport of ASH1 mRNA in S. cerevisiae. Since then, we expanded our research to neuronal mRNA-protein complexes and their specificity in binding. Two of these neuronal projects involve RNA-binding proteins that have been associated with neuronal disorders:
PURA Syndrome: PURA is an ubiquitously expressed protein with enrichment in the brain. Spontaneous mutations in its gene result in the rare neurodevelopmental disorder PURA Syndrome. We are closely interacting with the patient-centered PURA Syndrome Foundation to understand which molecular and cellular pathways are affected in this disorder and result in the patient's symptoms. Towards this goal, we combine our classical structural and biochemical approaches with cell-culture studies, including iPSC-based analyses, and various omics approaches.
Poly-glutamine diseases: These diseases are caused by pathological genomic expansions of disease-related genes that are translated into polyQ stretches. Proteins with such expanded polyQ stretches tend to form neurotoxic aggregates. Together with our collaboration partners, we are characterizing the therapeutic potential of a novel drug target towards a preventive treatment of affected patients in early stages of their disease.
研究兴趣
论文共 124 篇作者统计合作学者相似作者
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Marcel Proske,Robert Janowski,Sabrina Bacher,Hyun-Seo Kang,Thomas Monecke, Tony Köhler,Saskia Hutten, Jana Tretter, Anna Crois,Lena Molitor,Alejandro Varela-Rial,Roberto Fino,
eLife (2024)
ACTA CRYSTALLOGRAPHICA SECTION F-STRUCTURAL BIOLOGY COMMUNICATIONSno. Pt 2 (2024): 36-42
biorxiv(2024)
Annika Niedner-Boblenz,Thomas Monecke,Janosch Hennig,Melina Klostermann,Mario Hofweber,Elena Davydova,André P. Gerber,Irina Anosova, Wieland Mayer,Marisa Müller, Roland Heym,Robert Janowski,
crossref(2024)
Monika Witzenberger,Sandra Burczyk,David Settele, Wieland Mayer,Luisa M. Welp,Matthias Heiss,Mirko Wagner,Thomas Monecke,Robert Janowski,Thomas Carell,Henning Urlaub, Stefanie M. Hauck,
Nucleic Acids Research (2023)
biorxiv(2022)
crossref(2022)
Lena Molitor,Melina Klostermann,Sabrina Bacher,Juliane Merl-Pham, Nadine Spranger,Sandra Burczyk, Carolin Ketteler,Ejona Rusha,Daniel Tews,Anna Pertek, Marcel Proske,Anke Busch,
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