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1-11 of 11
Keywords: TDP-43
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Journal Articles
In collection:
Neurodegenerative Disorders
Eleni Christoforidou, Libby Moody, Greig Joilin, Fabio A. Simoes, David Gordon, Kevin Talbot, Majid Hafezparast
Journal:
Disease Models & Mechanisms
Dis Model Mech (2024) 17 (5): dmm050638.
Published: 29 May 2024
... 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...
Includes: Supplementary data
Journal Articles
Journal:
Disease Models & Mechanisms
Dis Model Mech (2024) 17 (3): dmm050473.
Published: 28 March 2024
... 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...
Includes: Supplementary data
Journal Articles
In collection:
Neurodegenerative Disorders
Journal:
Disease Models & Mechanisms
Dis Model Mech (2022) 15 (9): dmm049418.
Published: 13 September 2022
...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...
Includes: Supplementary data
Journal Articles
In collection:
Neuromuscular Disease
Miriam Pacetti, Laura De Conti, Luciano E. Marasco, Maurizio Romano, Mohammad M. Rashid, Martina Nubiè, Francisco E. Baralle, Marco Baralle
Journal:
Disease Models & Mechanisms
Dis Model Mech (2022) 15 (4): dmm049032.
Published: 29 April 2022
...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...
Includes: Supplementary data
Journal Articles
Caitlin S. Latimer, Jade G. Stair, Joshua C. Hincks, Heather N. Currey, Thomas D. Bird, C. Dirk Keene, Brian C. Kraemer, Nicole F. Liachko
Journal:
Disease Models & Mechanisms
Dis Model Mech (2022) 15 (4): dmm049323.
Published: 27 April 2022
...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...
Includes: Supplementary data
Journal Articles
In collection:
Neurodegenerative Disorders
Journal:
Disease Models & Mechanisms
Dis Model Mech (2021) 14 (2): dmm047548.
Published: 11 February 2021
...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...
Includes: Supplementary data
Journal Articles
Eileen Lynch, Theran Semrad, Vincent S. Belsito, Claire FitzGibbons, Megan Reilly, Koji Hayakawa, Masatoshi Suzuki
Journal:
Disease Models & Mechanisms
Dis Model Mech (2019) 12 (8): dmm039552.
Published: 16 August 2019
... 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...
Includes: Supplementary data
Journal Articles
In collection:
Neurodegenerative Disorders
Tongcui Jiang, Emily Handley, Mariana Brizuela, Edgar Dawkins, Katherine E. A. Lewis, Rosemary M. Clark, Tracey C. Dickson, Catherine A. Blizzard
Journal:
Disease Models & Mechanisms
Dis Model Mech (2019) 12 (5): dmm038109.
Published: 17 May 2019
... 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...
Includes: Supplementary data
Journal Articles
In collection:
Neurodegenerative Disorders
Journal:
Disease Models & Mechanisms
Dis Model Mech (2016) 9 (9): 1029–1037.
Published: 1 September 2016
...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...
Includes: Supplementary data
Journal Articles
Simona Langellotti, Valentina Romano, Giulia Romano, Raffaella Klima, Fabian Feiguin, Lucia Cragnaz, Maurizio Romano, Francisco E. Baralle
Journal:
Disease Models & Mechanisms
Dis Model Mech (2016) 9 (6): 659–669.
Published: 1 June 2016
...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...
Includes: Supplementary data
Journal Articles
Journal:
Disease Models & Mechanisms
Dis Model Mech (2014) 7 (10): 1175–1184.
Published: 1 October 2014
.... 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...