M06 Olesoxime Treatment Inhibits The Formation Of Mhtt Fragments Through Suppression Of Calpain Activity, And Leads To Behavioural And Neurological Improvements In The Bachd Rat

Autor: Olaf Riess, M Michaud, Bernd J. Pichler, LE Clemens, R Pruss, Jonasz J. Weber, Schamim H. Eckert, Janett Eckmann, Carsten Calaminus, Libo Yu-Taeger, TT Wlodkowski, Andreas Weiss, H Phuc Nguyen, Nicolas Casadei, Jcd Magg, Thierry Bordet, Gunter P. Eckert

Publikováno v: Journal of neurology, neurosurgery, and psychiatry 85(Suppl 1), A96-A96 (2014). doi:10.1136/jnnp-2014-309032.278

Background Proteolytic cleavage of the mutant huntingtin protein (mHtt) leads to toxic N-terminal mHtt fragments, which are known to disrupt mitochondrial function. Olesoxime, a small cholesterol-like molecule, targets mitochondria and has demonstrat

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::55f86f00ca5171a184e4a4df18b5150b
https://doi.org/10.1136/jnnp-2014-309032.278

B12 The influence of kalirin-7 deficiency on the phenotype of YAC128 mice

Autor: Olaf Riess, Alexandra Kelp, LE Clemens, Huu P. Nguyen, G Pahnke, Jcd Magg, R Mains, E Portal, M Schaller

Publikováno v: Journal of Neurology, Neurosurgery & Psychiatry. 83:A9.2-A9

Background The guanine-nucleotide exchange factor kalirin is a key regulator of spine morphogenesis and important for synaptic function and glutamatergic transmission. Kalirin-7 -knockout mice (Kal7KO) deficient for the most abundant isoform of kalir

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::a8067e5abc3368e447c0f428321102da
https://doi.org/10.1136/jnnp-2012-303524.28

Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3.

Autor: Harmuth T; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.; Centre for Rare Diseases, Tübingen, Germany.; Graduate School of Cellular Neuroscience, University of Tübingen, Tübingen, Germany., Prell-Schicker C; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany., Weber JJ; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.; Centre for Rare Diseases, Tübingen, Germany., Gellerich F; Department of Neurology, University Hospital Magdeburg, Magdeburg, Germany., Funke C; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany., Drießen S; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany., Magg JCD; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.; Centre for Rare Diseases, Tübingen, Germany., Krebiehl G; Center of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany., Wolburg H; Institute of Pathology and Neuropathology, University of Tübingen, Tübingen, Germany., Hayer SN; German Center for Neurodegenerative Diseases, Tübingen, Germany.; Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research and Center of Neurology, University of Tübingen, Tübingen, Germany., Hauser S; German Center for Neurodegenerative Diseases, Tübingen, Germany., Krüger R; Center of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.; Luxembourg Centre for Systems Biomedicine, University of Luxembourg, Centre Hospitalier de Luxembourg, Luxembourg, Luxembourg., Schöls L; German Center for Neurodegenerative Diseases, Tübingen, Germany.; Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research and Center of Neurology, University of Tübingen, Tübingen, Germany., Riess O; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.; Centre for Rare Diseases, Tübingen, Germany., Hübener-Schmid J; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.; Centre for Rare Diseases, Tübingen, Germany.

Publikováno v: Frontiers in molecular neuroscience [Front Mol Neurosci] 2018 Oct 10; Vol. 11, pp. 368. Date of Electronic Publication: 2018 Oct 10 (Print Publication: 2018).