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Keywords: Amyotrophic lateral sclerosis
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Journal Articles
Dis Model Mech (2021) 14 (2): dmm047548.
Published: 11 February 2021
... lateral sclerosis and frontotemporal dementia, using the eye as a model and demonstrated axonal cytoskeleton alterations. TDP-43 Amyotrophic lateral sclerosis Frontotemporal lobar degeneration Neurodegeneration Disease model Visual system Amyotrophic lateral sclerosis (ALS) and...
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 in the...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (7): dmm044867.
Published: 07 July 2020
... 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 impairment and muscular atrophy ( Deenen et al...
Journal Articles
Dis Model Mech (2020) 13 (2): dmm041947.
Published: 15 November 2019
... the microbiome, immune system and epigenetic marks, establishing a time line that may pinpoint biomarkers of ALS for earlier diagnosis and therapeutic intervention. Amyotrophic lateral sclerosis G93A Gut Neurodegeneration SOD1 Immunophenotype Amyotrophic lateral sclerosis (ALS) is a...
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: 02 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): 559–579.
Published: 01 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 (2017) 10 (5): 551–558.
Published: 01 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). The...
Journal Articles
Dis Model Mech (2016) 9 (9): 1029–1037.
Published: 01 September 2016
... 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...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2015) 8 (8): 831–842.
Published: 01 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: 01 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...