Age-dependent neurodegeneration and organelle transport deficiencies in mutant TDP43 patient-derived neurons are independent of TDP43 aggregation
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 S393L and G294V mutant spinal motor neuron culture...
Main Authors: | N. Kreiter, A. Pal, X. Lojewski, P. Corcia, M. Naujock, P. Reinhardt, J. Sterneckert, S. Petri, F. Wegner, A. Storch, A. Hermann |
---|---|
Format: | Article |
Language: | English |
Published: |
Elsevier
2018-07-01
|
Series: | Neurobiology of Disease |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S096999611830086X |
Similar Items
-
Transcriptomic Analysis of Zebrafish TDP-43 Transgenic Lines
by: Alexandra Lissouba, et al.
Published: (2018-12-01) -
Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation
by: Fernando Ormeño, et al.
Published: (2020-02-01) -
Physiological tissue-specific and age-related reduction of mouse TDP-43 levels is regulated by epigenetic modifications
by: Baralle, F.E, et al.
Published: (2022) -
Robustness and Vulnerability of the Autoregulatory System That Maintains Nuclear TDP-43 Levels: A Trade-off Hypothesis for ALS Pathology Based on in Silico Data
by: Akihiro Sugai, et al.
Published: (2018-02-01) -
Molecular Mechanisms Underlying TDP-43 Pathology in Cellular and Animal Models of ALS and FTLD
by: Alistair Wood, et al.
Published: (2021-04-01)