...Sophie E. Badger; Ian Coldicott; Ergita Kyrgiou-Balli; Adrian Higginbottom; Chloé Moutin; Kamallia Mohd Imran; John C. Day; Johnathan Cooper-Knock; Richard J. Mead; James J. P. Alix ABSTRACT C9orf72 -related amyotrophic lateral sclerosis (ALS)/frontotemporal dementia (FTD) has proven difficult...

... stress. We here identified tsRNA profiles within susceptible tissues in animal models of amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and Parkinson's disease (PD) to pinpoint disease-specific tsRNAs and those shared across neurodegenerative diseases. We performed small RNA...

...Eleni Christoforidou; Libby Moody; Greig Joilin; Fabio A. Simoes; David Gordon; Kevin Talbot; Majid Hafezparast ABSTRACT Evidence suggests the presence of microglial activation and microRNA (miRNA) dysregulation in amyotrophic lateral sclerosis (ALS), the most common form of adult motor neuron...

...Mara Prior-González; Rafael Lazo-Gómez; Ricardo Tapia ABSTRACT Motor neuron (MN) loss is the primary pathological hallmark of amyotrophic lateral sclerosis (ALS). Histone deacetylase 4 (HDAC4) is one of several factors involved in nerve–muscle communication during MN loss, hindering muscle...

...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...

... 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...

... 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...

...) 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...

... pathological protein in amyotrophic 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...

...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...

.../100015591 Amyotrophic Lateral Sclerosis Association 10.13039/100000971 18-IIA-401 National Science Foundation 10.13039/100000001 DGE 1418060 Neuromuscular diseases collectively affect 160 per 100,000 people worldwide and are generally characterized by progressive motor...

.... , 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 Banerjee , R. , Mosley , R. L...

...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...

...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...

... 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...

...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...

...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...

...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...

... 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...

...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...