Zobrazeno 1 - 10
of 65
pro vyhledávání: '"LE Clemens"'
Publikováno v:
Genes, Brain and Behavior. 13:305-321
Behavioral characterization is an important part of establishing novel animal models, but classical behavioral tests struggle to reveal conclusive results due to problems with both reproducibility and validity. On the contrary, automated homecage obs
Publikováno v:
Rare Diseases
Olesoxime, a small molecule drug candidate, has recently attracted attention due to its significant beneficial effects in models of several neurodegenerative disorders including Huntington's disease. Olesoxime's neuroprotective effects have been assu
Autor:
LE Clemens, Olaf Riess, Huu Phuc Nguyen, Amelie J. Müller, Silke Metzger, Tassula Proikas-Cezanne, Petra Fallier-Becker, Carolin Walter
Publikováno v:
Neuropharmacology. 108
The expansion of a polyglutamine repeat in huntingtin (HTT) causes Huntington disease (HD). Although the exact pathogenesis is not entirely understood, mutant huntingtin (mHTT) causes disruption of various cellular functions, formation of aggregates
Autor:
Kim A. Verwaest, Trung N. Vu, LE Clemens, Kris Laukens, Björn Van Gasse, José C. Martins, Annemie Van Der Linden, Roger Dommisse, Huu P. Nguyen
Publikováno v:
Biochimica et biophysica acta : molecular basis of disease
Huntington disease (HD) is a hereditary brain disease. Although the causative gene has been found, the exact mechanisms of the pathogenesis are still unknown. Recent investigations point to metabolic and energetic dysfunctions in HD neurons. Both uni
Autor:
Gunter P. Eckert, LE Clemens, Jonasz J. Weber, Carsten Calaminus, Andreas Weiss, E Jansson, Libo Yu-Taeger, Tanja T. Wlodkowski, Thierry Bordet, Rebecca M. Pruss, Olaf Riess, Bernd J. Pichler, Janett Gaca, Janine Magg, Huu P. Nguyen, Schamim H. Eckert, Magali Michaud
Publikováno v:
Brain : a journal of neurology. 138(Pt 12)
Huntington's disease is a fatal human neurodegenerative disorder caused by a CAG repeat expansion in the HTT gene, which translates into a mutant huntingtin protein. A key event in the molecular pathogenesis of Huntington's disease is the proteolytic
Autor:
Stephanie Hagl, LE Clemens, Walter E. Müller, Janett Eckmann, Rebecca M. Pruss, Kristina Leuner, Gunter P. Eckert, Huu P. Nguyen, Thierry Bordet, Schamim H. Eckert, Libo Yu-Taeger
Publikováno v:
Molecular neurobiology. 50(1)
Huntington disease (HD) is a fatal neurodegenerative disorder caused by a CAG repeat expansion in exon 1 of the huntingtin gene (HTT). One prominent target of the mutant huntingtin protein (mhtt) is the mitochondrion, affecting its morphology, distri
Autor:
X. William Yang, Xiaofeng Gu, Libo Yu-Taeger, Huu Phuc Nguyen, Silke Metzger, Carsten Calaminus, LE Clemens, David Howland, Bernd J. Pichler, Elisabeth Petrasch-Parwez, Olaf Riess, Larry Park, Adriana Redensek, Alexander P. Osmand
Publikováno v:
The Journal of neuroscience : the official journal of the Society for Neuroscience. 32(44)
Huntington disease (HD) is an inherited progressive neurodegenerative disorder, characterized by motor, cognitive, and psychiatric deficits as well as neurodegeneration and brain atrophy beginning in the striatum and the cortex and extending to other
Autor:
Stephan M. Huber, Midea Malena Ortiz-Rios, Lukas Klumpp, Olaf Riess, Jonasz J. Weber, Huu P. Nguyen, Don C Lo, LE Clemens, Linda S. Kaltenbach
Publikováno v:
Journal of Neurology, Neurosurgery & Psychiatry. 87:A96.3-A97
Background A long-term treatment study of BACHD rats with olesoxime, a mitochondria-targeting drug, revealed beneficial effects on specific behavioural and neuropathological phenotypes. On the molecular level, olesoxime suppressed the activation of c
Autor:
Huu P. Nguyen, Jonasz J. Weber, Carolin Walter, Elisabeth Singer, LE Clemens, Ulrike A. Mau-Holzmann, Ann-Christin Krahl, Nadine Rischert
Publikováno v:
Journal of Neurology, Neurosurgery & Psychiatry. 87:A27.3-A28
Background The ST Hdh cell lines are immortalised striatal precursor cells from wild type and Hdh Q111 knock-in mice and are commonly used to study the molecular aspects of HD. Morphological differences between the wild type and mutant cell lines exi
Autor:
LE Clemens, Stefanie Flunkert, Birgit Hutter-Paier, Tiberiu Loredan Stan, Huu Phuc Nguyen, Robert Wronski, Stephan Kurat
Publikováno v:
Journal of Neurology, Neurosurgery & Psychiatry. 87:A28.3-A29
Background Huntington’s disease (HD) is an inherited, autosomal, neurodegenerative disease. Symptoms of HD patients often include motor, emotional and cognitive deficits. BACHD rats overexpress the full length human mutant huntingtin gene with 97 a