... disease. However, few studies have investigated whether the miRNA dysregulation originates from microglia. Furthermore, TDP-43 (encoded by TARDBP ), involved in miRNA biogenesis, aggregates in tissues of ∼98% of ALS cases. Thus, this study aimed to determine whether expression of the ALS-linked TDP-43...

... in vivo and found that Dendra2::tau turned over faster than the relatively stable Dendra2. Furthermore, Dendra2::tau turnover was dependent on the protein expression level and independent of co-expression with human TDP-43 (officially known as TARDBP), an aggregating protein interacting with pathological...

...Maize C. Cao; Emma L. Scotter ABSTRACT TDP-43 proteinopathy is the major pathology in amyotrophic lateral sclerosis (ALS) and tau-negative frontotemporal dementia (FTD). Mounting evidence implicates loss of normal TDP-43 RNA-processing function as a key pathomechanism. However, the RNA targets...

...Miriam Pacetti; Laura De Conti; Luciano E. Marasco; Maurizio Romano; Mohammad M. Rashid; Martina Nubiè; Francisco E. Baralle; Marco Baralle ABSTRACT The cellular level of TDP-43 (also known as TARDBP) is tightly regulated; increases or decreases in TDP-43 have deleterious effects in cells...

...Caitlin S. Latimer; Jade G. Stair; Joshua C. Hincks; Heather N. Currey; Thomas D. Bird; C. Dirk Keene; Brian C. Kraemer; Nicole F. Liachko ABSTRACT Although amyloid β (Aβ) and tau aggregates define the neuropathology of Alzheimer's disease (AD), TDP-43 has recently emerged as a co-morbid pathology...

...Rachel Atkinson; Jacqueline Leung; James Bender; Matthew Kirkcaldie; James Vickers; Anna King ABSTRACT Mislocalization of the TAR DNA-binding protein 43 (TDP-43; encoded by TARDBP ) from the nucleus to the cytoplasm is a common feature of neurodegenerative conditions such as amyotrophic lateral...

... in the expression of mitochondrial genes and a susceptibility to oxidative stress, indicating that mitochondrial dysfunction may be a critical feature of C9-ALS skeletal muscle pathology. Finally, the C9-ALS myocytes had increased expression and aggregation of TDP-43. Together, these data show that skeletal muscle...

... models. Recent studies propose an important role for TAR DNA-binding protein 43 (TDP-43), the mislocalization and aggregation of which are key pathological features of ALS. However, the relationship between ALS-linked TDP-43 mutations, excitability and synaptic function is not fully understood. Here, we...

...Karen S. Coughlan; Luise Halang; Ina Woods; Jochen H. M. Prehn ABSTRACT Transgenic transactivation response DNA-binding protein 43 (TDP-43) mice expressing the A315T mutation under control of the murine prion promoter progressively develop motor function deficits and are considered a new model...

...Simona Langellotti; Valentina Romano; Giulia Romano; Raffaella Klima; Fabian Feiguin; Lucia Cragnaz; Maurizio Romano; Francisco E. Baralle ABSTRACT Transactive response DNA-binding protein 43 kDa (TDP-43, also known as TBPH in Drosophila melanogaster and TARDBP in mammals) is the main protein...

.... FUS Hsp104 TDP-43 α-synuclein Disaggregase Neurodegeneration Fatal neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), Parkinson disease (PD), Huntington disease (HD) and Alzheimer disease (AD) are classified as protein-misfolding disorders ( Cushman et al., 2010...