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Keywords: Amyotrophic lateral sclerosis
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Journal Articles
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
Dis Model Mech (2022) 15 (8): dmm049059.
Published: 29 August 2022
... traits of cell senescence in the lumbar spinal cord, with selective sensitivity to senolytic treatments. Amyotrophic lateral sclerosis Navitoclax Senolytic Neuroinflammation Therapy Cell cycle Cryptic exon Instituto de Salud Carlos III http://dx.doi.org/10.13039/501100004587 PI...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2022) 15 (4): dmm049032.
Published: 29 April 2022
... different ages. Furthermore, epigenetic control was observed in mouse and human cultured cell lines. In amyotrophic lateral sclerosis, the formation of TDP-43-containing brain inclusions removes functional protein from the system. This phenomenon is continuous but compensated by newly synthesized protein...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2021) 14 (11): dmm049092.
Published: 1 December 2021
...) and amyotrophic lateral sclerosis (ALS). This repeat expansion can be translated into dipeptide repeat proteins (DPRs), and distribution of the poly-GR DPR correlates with neurodegeneration in postmortem C9FTD/ALS brains. Here, we assessed poly-GR toxicity in zebrafish embryos, using an annexin A5-based...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2021) 14 (2): dmm047548.
Published: 11 February 2021
... , G. , Korb , E. , Rao , E. J. , Wu , J. Y. and Finkbeiner , S. ( 2010 ). Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis . J. Neurosci. 30 , 639 - 649 . 10.1523/JNEUROSCI.4988...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (10): dmm045732.
Published: 30 October 2020
...Cristina Ruiz-Ruiz; Nuria García-Magro; Pilar Negredo; Carlos Avendaño; Anindya Bhattacharya; Marc Ceusters; Antonio G. García ABSTRACT Neuroinflammation is one of the main physiopathological mechanisms of amyotrophic lateral sclerosis (ALS), produced by the chronic activation of microglia...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (7): dmm044867.
Published: 7 July 2020
... neuromuscular disease models. Skeletal muscle Motor neurons Amyotrophic lateral sclerosis Induced pluripotent stem cells Tissue engineering Microfluidic devices Neuromuscular diseases collectively affect 160 per 100,000 people worldwide and are generally characterized by progressive motor...
Journal Articles
Dis Model Mech (2020) 13 (2): dmm041947.
Published: 15 November 2019
... Atkin , J. D. , Scott , R. L. , West , J. M. , Lopes , E. , Quah , A. K. and Cheema , S. S. ( 2005 ). Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis . Neuromuscul. Disord.   15 , 377 - 388 . 10.1016/j.nmd.2005.02.005...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2019) 12 (8): dmm039552.
Published: 16 August 2019
...Eileen Lynch; Theran Semrad; Vincent S. Belsito; Claire FitzGibbons; Megan Reilly; Koji Hayakawa; Masatoshi Suzuki ABSTRACT Amyotrophic lateral sclerosis (ALS) is a late-onset neuromuscular disease with no cure and limited treatment options. Patients experience a gradual paralysis leading to death...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2019) 12 (8): dmm040238.
Published: 13 August 2019
...Martin Crivello; Marion C. Hogg; Elisabeth Jirström; Luise Halang; Ina Woods; Megan Rayner; Karen S. Coughlan; Sebastian A. Lewandowski; Jochen H. M. Prehn ABSTRACT Amyotrophic lateral sclerosis (ALS) presents a poorly understood pathogenesis. Evidence from patients and mutant SOD1 mouse models...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2019) 12 (1): dmm037424.
Published: 2 January 2019
... mutation, as well as from the specific changes to the gene of interest. Here, we review the current range of mouse models with mutations in genes causative for the human neurodegenerative disease amyotrophic lateral sclerosis. We focus on the two main types of available mutants: transgenic mice and those...
Journal Articles
Dis Model Mech (2018) 11 (4): dmm031997.
Published: 16 April 2018
...Shruthi Shanmukha; Gayathri Narayanappa; Atchayaram Nalini; Phalguni Anand Alladi; Trichur R. Raju ABSTRACT Skeletal muscle atrophy is the most prominent feature of amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease of motor neurons. However, the contribution of skeletal...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (5): 551–558.
Published: 1 May 2017
...María Fernández-Trapero; Francisco Espejo-Porras; Carmen Rodríguez-Cueto; Joan R. Coates; Carmen Pérez-Díaz; Eva de Lago; Javier Fernández-Ruiz ABSTRACT Targeting of the CB 2 receptor results in neuroprotection in the SOD1 G93A mutant mouse model of amyotrophic lateral sclerosis (ALS...
Journal Articles
Dis Model Mech (2017) 10 (5): 559–579.
Published: 1 May 2017
...Kyoung-in Cho; Dosuk Yoon; Sunny Qiu; Zachary Danziger; Warren M. Grill; William C. Wetsel; Paulo A. Ferreira ABSTRACT The pathogenic drivers of sporadic and familial motor neuron disease (MND), such amyotrophic lateral sclerosis (ALS), are unknown. MND impairs the Ran GTPase cycle, which controls...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2016) 9 (9): 1029–1037.
Published: 1 September 2016
... for the study of amyotrophic lateral sclerosis (ALS); however, premature sudden death resulting from intestinal obstruction halts disease phenotype progression in 100% of C57BL6/J congenic TDP-43 A315T mice. Similar to our recent results in SOD1 G93A mice, TDP-43 A315T mice fed a standard pellet diet showed...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2015) 8 (8): 831–842.
Published: 1 August 2015
...Xiaohua Xu; Aleksandar Denic; Luke R. Jordan; Nathan J. Wittenberg; Arthur E. Warrington; Bharath Wootla; Louisa M. Papke; Laurie J. Zoecklein; Daehan Yoo; Jonah Shaver; Sang-Hyun Oh; Larry R. Pease; Moses Rodriguez ABSTRACT Amyotrophic lateral sclerosis (ALS) is a devastating, fatal neurological...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2015) 8 (3): 215–224.
Published: 1 March 2015
...Catherine A. Blizzard; Katherine A. Southam; Edgar Dawkins; Katherine E. Lewis; Anna E. King; Jayden A. Clark; Tracey C. Dickson There is a desperate need for targeted therapeutic interventions that slow the progression of amyotrophic lateral sclerosis (ALS). ALS is a disorder with heterogeneous...