Age-dependent neurodegeneration and organelle transport deficiencies in mutant TDP43 patient-derived neurons are independent of TDP43 aggregation

Autor: N. Kreiter, A. Pal, X. Lojewski, P. Corcia, M. Naujock, P. Reinhardt, J. Sterneckert, S. Petri, F. Wegner, A. Storch, A. Hermann

Publikováno v: Neurobiology of Disease, Vol 115, Iss , Pp 167-181 (2018)

TAR DNA-binding protein 43 (TDP43) plays a significant role in familiar and sporadic amyotrophic lateral sclerosis (ALS). The diverse postulated mechanisms by which TDP43 mutations cause the disease are not fully understood. Human wildtype and TDP43

Externí odkaz: https://doaj.org/article/905590cbf89348f0af641db3b2465006

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Stepwise acquirement of hallmark neuropathology in FUS-ALS iPSC models depends on mutation type and neuronal aging.

Autor: Japtok J; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany., Lojewski X; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany., Naumann M; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany., Klingenstein M; Institute of Neuroanatomy, Eberhard Karls University of Tübingen, Germany., Reinhardt P; Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany., Sterneckert J; Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany., Putz S; Institute of Anatomy and Cell Biology, University of Ulm, Germany., Demestre M; Institute of Anatomy and Cell Biology, University of Ulm, Germany., Boeckers TM; Institute of Anatomy and Cell Biology, University of Ulm, Germany., Ludolph AC; Department of Neurology, University Ulm, Germany., Liebau S; Institute of Neuroanatomy, Eberhard Karls University of Tübingen, Germany., Storch A; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany; German Center for Neurodegenerative Diseases (DZNE), 01307 Dresden, Germany; Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany., Hermann A; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany; German Center for Neurodegenerative Diseases (DZNE), 01307 Dresden, Germany. Electronic address: Andreas.Hermann@uniklinikum-dresden.de.

Publikováno v: Neurobiology of disease [Neurobiol Dis] 2015 Oct; Vol. 82, pp. 420-429. Date of Electronic Publication: 2015 Aug 04.