Results 111 to 120 of about 95,709 (300)

A case of motor neuron involvement in Gaucher disease

Molecular Genetics and Metabolism Reports, 2019
Gaucher disease (GD) is a genetic disorder characterized by an accumulation of glucosylceramide in cells in the monocyte-macrophage system. We describe a case of a 33-year-old man with a previous diagnosis of type 3 GD who displayed a progressive ...
V. Pozzilli, F. Giona, M. Ceccanti, C. Cambieri, V. Frasca, E. Onesti, L. Libonati, S. Di Bari, I. Fiorini, L. Cardarelli, M. Santopietro, M. Inghilleri   +11 more
doaj   +1 more source

Early exposure to environmental toxin contributes to neuronal vulnerability and axonal pathology in a model of familial ALS [PDF]

, 2011
Adult onset amyotrophic lateral sclerosis (ALS) arises due to progressive and irreversible functional deficits to the central nervous system, specifically the loss of motor neurons.
Christopher A. Shaw, Grace Lee
core   +1 more source

Bilirubin as a Modulator of WNK1 Protein Signaling: Implications for Neuroinflammatory Diseases

Advanced Science, EarlyView.
ABSTRACT Previously regarded merely as a potentially harmful waste product of heme catabolism, bilirubin has now emerged as a pleiotropic molecule with potent antioxidant, anti‐inflammatory, and hormone‐like properties. Recent findings have revealed protective effects against cardiovascular, metabolic, autoimmune, and neoplastic diseases, as well as ...
Sri Jayanti, Libor Vítek, Silvia Gazzin, Claudio Tiribelli   +3 more
wiley   +1 more source

Endoplasmic Reticulum Geometry Dictates Neuronal Bursting via Calcium Store Refill Rates and Exposes Selective Neuronal Vulnerability

Advanced Science, EarlyView.
The ER's continuous tubular network is maintained by ER‐shaping proteins whose mutation or dysregulation contributes to neurodegenerative diseases. Here, we show that ER morphology sets the speed of Ca2+ store replenishment between firing events. Disrupting ER continuity slows intra‐ER Ca2+ redistribution from extracellular refill (SOCE) sites, driving
Valentina Davi, Pierre Parutto, Yuyi Zhang, Tasuku Konno, Cecile Crapart, Raquel Pereira, John P. Franklin, Mosab Ali Awadelkareem, Daniel C Maddison, Michael J. Devine, Edgar R. Gomes, Joseph Chambers, Elena Koslover, Edward Avezov   +13 more
wiley   +1 more source

Engineering Approaches to Modify Immunomodulatory Functions of Mesenchymal Stromal Cells (MSCs): Tissue Regeneration and Clinical Application

Advanced Science, EarlyView.
Mesenchymal stromal cells (MSCs) show promise for treating immune‐related disorders through immunomodulation and tissue regeneration. This review gives a brief overview of current clinical approval of MSC therapies. It also discussed how bioengineering, including genetic modification, biomaterial delivery, extracellular vesicles, and iPSC‐derived MSCs,
Sichen Yang, Kejia Li, Ning Wang, Zhen Li, Zhiyong Zhang, Rocky S. Tuan, Yangzi Jiang   +6 more
wiley   +1 more source

Frontostriatal deficit in Motor Neuron Disease/Amyotrophic Lateral Sclerosis (MND/ALS) [PDF]

, 2003
So far, cognitive derangements in MND/ALS have not been widely studied. Nevertheless, it seems that in subgroups of patients cognitive functions are impaired in different degree, so that often at least two sub-types of the syndrome are reported: Motor ...
Bongioanni, Dr. P, Buoiano, Dr. G, Carboncini, Dr. MC, Magoni, Dr. M, Rossi, Dr. B   +4 more
core  

The Emerging Parkinson's Disease Oxylipin‐Ome

Advanced Science, EarlyView.
ABSTRACT Parkinson Disease (PD) is increasingly considered a proteinopathy and lipidopathy. This proteinopathy+lipidopathy paradigm has been further refined to a fatty acid (FA)‐opathy, centering dysregulated FA metabolism as fundamental in PD lipid dysfunction.
Julia C. Kelliher, Saranna Fanning
wiley   +1 more source

Comparison of Movement Related Cortical Potential in healthy people and Amyotrophic Lateral Sclerosis patients

Frontiers in Neuroscience, 2013
Objective: To understand the brain motor functions and neurophysiological changes due to motor disorder by comparing electroencephalographic data between healthy people and amyotrophic lateral sclerosis (ALS) patients.
Ying eGu, Dario eFarina, Ander Ramos eMurguialday, Ander Ramos eMurguialday, Kim eDremstrup, Niels eBirbaumer, Niels eBirbaumer   +6 more
doaj   +1 more source

Dysphagia in Amyotrophic Lateral Sclerosis (ALS)

Acta Logopaedica
Around 80 % of ALS patients suffer from dysphagia, which increases the risk of malnutrition and pneumonia, and correlates with death. To diminish that risk, it is important to involve patients in their dysphagia care to be able to introduce interventions in time. However, Swedish ALS teams lack national guidelines for how to involve patients.
Ann-Sofie Eriksson, Kerstin Johansson
openaire   +1 more source

Orthogonal Ionic Liquid‐Based Extraction Strategy Enables Amyloid‐Specific Profiling of Aggregate Proteome

Advanced Science, EarlyView.
An orthogonal ionic‐liquid extraction (Orth‐iEA) enables selective isolation of amyloid fibrils. TMGBF4 disrupts hydrogen‐bonded β‐sheet networks to solubilize amyloid aggregates, whereas C12ImCl interacts with hydrophobic regions of non‐amyloid proteins.
Shiying Zheng, Ye Liu, Bowen Zhong, Huiying Chu, Zhou Gong, Baofeng Zhao, Mengchun Cheng, Zhen Liang, Yukui Zhang, Qun Zhao, Lihua Zhang   +10 more
wiley   +1 more source