Results 61 to 70 of about 242,580 (385)

Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease [PDF]

, 2018
Human Ag R (HuR) is an RNA binding protein in the ELAVL protein family. To study the neuron-specific function of HuR, we generated inducible, neuron-specific HuR-deficient mice of both sexes.
Bai, Ying, Chen, Xing, Hamilton, Thomas A., Herjan, Tomasz, Kokiko-Cochran, Olga N., Lamb, Bruce, Lee, Yu-Shang, Li, Xiao, Lin, Ching-Yi, Sun, Kevin, Zhou, Gao   +10 more
core   +1 more source

Amyotrophic Lateral Sclerosis Prevalence Projection in 2040: A Less Rare Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To project ALS prevalence across multiple countries through 2040, accounting for both population aging and increased survival. Methods Data from the Piemonte and Valle d'Aosta ALS register (PARALS) was used to estimate the trends in incidence and prevalence from 2005 to 2019. Survival trends over this period were also assessed.
Rosario Vasta, Stefano Callegaro, Antonio Canosa, Umberto Manera, Maurizio Grassano, Francesca Palumbo, Sara Cabras, Enrico Matteoni, Francesca Di Pede, Filippo De Mattei, Salvatore Tafaro, Neil M. Thakur, Ryan Grosenick, Fabiola De Marchi, Letizia Mazzini, Cristina Moglia, Andrea Calvo, Kuldip D. Dave, Adriano Chiò   +18 more
wiley   +1 more source

Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration

Neural Regeneration Research, 2022
Amyotrophic lateral sclerosis is a relentlessly progressive multi-system condition. The clinical picture is dominated by upper and lower motor neuron degeneration, but extra-motor pathology is increasingly recognized, including cerebellar pathology. Post-
Rangariroyashe H Chipika, Grainne Mulkerrin, Pierre-François Pradat, Aizuri Murad, Fabrice Ango, Cédric Raoul, Peter Bede   +6 more
doaj   +1 more source

Tremor in motor neuron disease may be central rather than peripheral in origin [PDF]

, 2018
BACKGROUND AND PURPOSE: Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post-mortem observations have revealed considerable variability in the phenotype.
Balint, B, Batla, A, Berardelli, A, Bhatia, K P, Ciocca, M, Latorre, A, Rocchi, L, Rothwell, J C, Sidle, K, Stamelou, M   +9 more
core   +2 more sources

Plasma Proteomic Signatures for Alzheimer's Disease: Comparable Accuracy to ATN Biomarkers and Cross‐Platform Validation

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Background There is growing recognition of the potential of plasma proteomics for Alzheimer's Disease (AD) risk assessment and disease characterization. However, differences between proteomics platforms introduce uncertainties regarding cross‐platform applicability.
Manyue Hu, Oliver Robinson, Christina M. Lill, Anna Matton, Raquel Puerta, Pilar Sanz, Merce Boada, Agustín Ruiz, Lefkos Middleton, for the Alzheimer's Disease Neuroimaging Initiative   +9 more
wiley   +1 more source

OSMR is a potential driver of inflammation in amyotrophic lateral sclerosis

Neural Regeneration Research
Amyotrophic lateral sclerosis is a neurodegenerative disease, and the molecular mechanism underlying its pathology remains poorly understood. However, inflammation is known to play an important role in the development of this condition.
Wenzhi Chen, Shishi Jiang, Shu Li, Cheng Li, Renshi Xu   +4 more
doaj   +1 more source

ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cells- derived motoneurons [PDF]

, 2015
Patient-derived induced Pluripotent Stem Cells (iPSCs) provide an opportunity to study human diseases mainly in those cases where no suitable model systems are available.
Bozzoni, Irene, Caliendo, Virginia, Calvo, Andrea, Chiò, Adriano, DE SANTIS, Riccardo, Laneve, Pietro, Lenzi, Jessica, Morlando, Mariangela, Rosa, Alessandro, Turris, Valeria de   +9 more
core   +4 more sources

Longitudinal biomarkers in amyotrophic lateral sclerosis

Annals of Clinical and Translational Neurology, 2020
To investigate neurodegenerative and inflammatory biomarkers in people with amyotrophic lateral sclerosis (PALS), evaluate their predictive value for ALS progression rates, and assess their utility as pharmacodynamic biomarkers for monitoring treatment ...
F. Huang, Yuda Zhu, Jennifer Hsiao‐Nakamoto, Xinyan Tang, J. Dugas, M. Moscovitch-Lopatin, J. Glass, Robert H. Brown, S. Ladha, D. Lacomis, Jeffrey M Harris, K. Scearce-Levie, Carole Ho, R. Bowser, J. Berry   +14 more
semanticscholar   +1 more source

Acoustic Measures Capture Speech Dysfunction in Spinocerebellar Ataxia

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Spinocerebellar ataxias (SCA) are hereditary cerebellar degenerative disorders with a common feature of dysarthria, involving impaired phonatory and articulatory control of speech, thereby affecting social communication. In this study, we investigated whether acoustic measures could objectively measure speech dysfunction and identify
Zena Fadel, Charlotte Hennessey, Hannah Lee, Pia Parekh, Sheng‐Han Kuo, Ami Kumar   +5 more
wiley   +1 more source

Real-world evidence of riluzole effectiveness in treating amyotrophic lateral sclerosis

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Objective To compare the effect of riluzole on median survival in population studies of patients with amyotrophic lateral sclerosis (ALS) with that observed in clinical trials.
Jinsy A. Andrews, C. Jackson, T. Heiman-Patterson, P. Bettica, B. Brooks, E. Pioro   +5 more
semanticscholar   +1 more source