Translational Protein Biochemistry
Prof. Dr. Tobias M. Boeckers
Group Leader
Albert Einstein Alle 11
89081 Ulm

tobias.boeckers@dzne.de
 +49 731 500 232 20

Areas of investigation/research focus

Neurodegenerative diseases such as frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS) or Huntington's disease are associated with selective loss of certain neuron populations and thus show a specific pattern of symptoms. Over the past years especially, disease genes have been successfully identified and been neuropathologically described. In particular, our group has used motoneurons and muscle cells from patient iPS cells to reveal cellular and molecular pathomechanisms for these diseases and to create a compound screening platform.

 more Infos

In the context of the DZNE cooperation group, the main focus will now be on validating the expression of relevant proteins that have been shown to be involved in the pathogenesis of neurodegeneration, in human tissue ("construct validity"). More precisely, proteins or pathway components involved in disease development or molecular therapeutic targets found in mouse models (but also in other models, e.g. Drosophila, C. elegans) are to be analyzed -prior to human studies- with respect to their expression in phylogenetically young parts of the human brain (those affected in ALS and FTD). The Braak collection, which has been established for decades, as well as the human tissue collection of the Institute of Anatomy and Cell Biology provide well-characterized brain tissue for examination. Furthermore, it will be necessary to intensify research efforts under the heading "Neurodegeneration as Synaptopathy" and related implications for new therapeutic approaches. In continuation of the successful work in the virtual Helmholtz Center in Ulm, especially the role of postsynaptic density (PSD) proteins in neurodegenerative diseases will be further investigated.

Key Publications

Picchiarelli G, Demestre M, Zuko A, Been M, Higelin J, Dieterlé S, Goy MA, Mallik M, Sellier C, Scekic-Zahirovic J, Zhang L, Rosenbohm A, Sijlmans C, Aly A, Mersmann S, Sanjuan-Ruiz I, Hübers A, Messaddeq N, Wagner M, van Bakel N, Boutillier AL, Ludolph A, Lagier-Tourenne C, Boeckers TM, Dupuis L, Storkebaum E. FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis. Nat Neurosci. 2019 Oct 07; doi: 10.1038/s41593-019-0498-9
Demestre M, Orth M, Foehr KJ, Achberger K, Ludolph AC, Liebau St, Boeckers TM. Formation and characterisation of neuromuscular junctions between hiPSc derived motoneurones and myotubes. Stem Cell Research. 2015 Sep 01; 15:328-336. doi: 10.1016/j.scr.2015.07.005
Schoen M, Reichel J, Demestre M, Putz St, Deshpande D, Proepper Ch, Liebau St, Schmeisser MJ, Ludolph AC, Michaelis J, Boeckers TM. Super-resolution microscopy reveals presynaptic localization of the ALS / FTD related protein FUS in hippocampal neurons. Front Cell Neurosci. 2016 Jan 12; 9:496. doi: 10.3389/fncel.2015.00496
Higelin J, Demestre M, Putz St, Lutz AK, Bausinger J, Hübers AK, Klingenstein M, Barbi G, Liebau St, Speit G, Hermann A, Ludolph AC, Boeckers TM. FUS mislocalisation and vulnerability to DNA damage in ALS patients derived hiPSCs and aging motoneurons. Front Cell Neuroscience. 2016 Dec 26; 10:290. doi: 10.3389/fncel.2016.00290
Naumann M, Pal A, Goswami A, Lojewski X, Japtok J, Vehlow A, Naujock M, Günther R, Jin M, Stanslowsky N, Reinhardt P, Sterneckert J, Frickenhaus M, Pan-Montojo F, Storkebaum E, Poser I, Freischmidt A, Weishaupt J, Holzmann KH, Troost D, Ludolph A, Boeckers TM, Liebau St, Petri S, Cordes N, Hyman A, Wegner F, Stephan G, Weis J, Storch A, and Hermann A. Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and aggregation formation. Nature Communication. 2018 Jan 23; 9:335. doi: 10.1038/s41467-017-02299-1

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