Results 71 to 80 of about 49,177 (260)

C9ORF72 in Japanese amyotrophic lateral sclerosis (ALS)

Rinsho Shinkeigaku, 2013
Recently, C9orf72 hexanucleotide (GGGGCC) repeat expansion in intron 1 was reported to be the most common cause of sporadic and familial amyotrophic lateral sclerosis (ALS)/frontotemporal dementia (FTD) in the Caucasian population. The frequency of the intronic repeat expansion is up to 21%-57% in familial ALS and 3%-21% in sporadic ALS.In the Japanese
openaire   +3 more sources

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

Enhancing Maturation of Human Neuromuscular Organoids via Electrical Stimulation

Advanced Science, EarlyView.
A framework for on‐demand and non‐invasive exposure of human neuromuscular organoids (NMOs) to electrical stimuli is established to promote their maturation. The robustness and effectiveness of different stimulation regimes are evaluated via thorough characterization of organoid tissue structure and contraction capacity. Chronic electrical stimulation,
Chrysanthi‐Maria Moysidou, Inês Afonso Martins, Ismail Amr El‐Shimy, Iacopo Bicci, Donatella Cea, Christina Bukas, Isra Mekki, Mara‐Camelia Rusu, Aylin Nebol, Ines Lahmann, Marie Piraud, Enrico Klotzsch, Mina Gouti   +12 more
wiley   +1 more source

A small-molecule inhibitor of SOD1-Derlin-1 interaction ameliorates pathology in an ALS mouse model

Nature Communications, 2018
Amyotrophic lateral sclerosis (ALS) is a neurological disease that leads to loss of voluntary muscle movement. Here, the authors screen for molecules that disrupt interaction between SOD1, a protein linked to ALS, and Derlin-1, and find an inhibitor that
Naomi Tsuburaya, Kengo Homma, Tsunehiko Higuchi, Andrii Balia, Hiroyuki Yamakoshi, Norio Shibata, Seiichi Nakamura, Hidehiko Nakagawa, Shin-ichi Ikeda, Naoki Umezawa, Nobuki Kato, Satoshi Yokoshima, Masatoshi Shibuya, Manabu Shimonishi, Hirotatsu Kojima, Takayoshi Okabe, Tetsuo Nagano, Isao Naguro, Keiko Imamura, Haruhisa Inoue, Takao Fujisawa, Hidenori Ichijo   +21 more
doaj   +1 more source

Mutant FUS and ELAVL4 (HuD) Aberrant Crosstalk in Amyotrophic Lateral Sclerosis

Cell Reports, 2019
Summary: Amyotrophic lateral sclerosis (ALS) has been genetically linked to mutations in RNA-binding proteins (RBPs), including FUS. Here, we report the RNA interactome of wild-type and mutant FUS in human motor neurons (MNs).
Riccardo De Santis, Vincenzo Alfano, Valeria de Turris, Alessio Colantoni, Laura Santini, Maria Giovanna Garone, Giuseppe Antonacci, Giovanna Peruzzi, Emma Sudria-Lopez, Emanuel Wyler, Jasper J. Anink, Eleonora Aronica, Markus Landthaler, R. Jeroen Pasterkamp, Irene Bozzoni, Alessandro Rosa   +15 more
doaj   +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

Early Dropped Head Syndrome In A Case With Amyotrophic Lateral Sclerosis

Annals of Indian Academy of Neurology, 2005
We Report a case of "Dropped head syndrome" [DHS] due to Amyotrophic lateral sclerosis [ALS]. A thirty seven year old gentleman was admitted with progressive limb and bulbar weakness of 10 months duration.
Shanbhogue K R, Rajesh S, Durairaj R, Mugundan K, Balasubramanian S, Gobinathan S, Velusamy S   +6 more
doaj  

Antiviral Immune Response as a Trigger of FUS Proteinopathy in Amyotrophic Lateral Sclerosis

Cell Reports, 2019
Summary: Mutations in the FUS gene cause familial amyotrophic lateral sclerosis (ALS-FUS). In ALS-FUS, FUS-positive inclusions are detected in the cytoplasm of neurons and glia, a condition known as FUS proteinopathy.
Tatyana A. Shelkovnikova, Haiyan An, Lucy Skelt, John S. Tregoning, Ian R. Humphreys, Vladimir L. Buchman   +5 more
doaj   +1 more source

Smart Nanotechnologies for Multimodal Neuromodulation and Brain Interfacing

Advanced Science, EarlyView.
Recent advances in smart nanotechnologies are expanding the toolbox for brain interfacing, from wireless neuromodulation and high‐resolution sensing to targeted delivery within the central nervous system. By combining responsive nanomaterials with bioinspired design, these platforms enable multimodal interactions with neurons and glia, while also ...
Tommaso Curiale, Marie Celine Lefevre, Alessio Carmignani, Maria Cristina Ceccarelli, Matteo Battaglini, Attilio Marino, Gianni Ciofani   +6 more
wiley   +1 more source

Amyotrophic Lateral Sclerosis (ALS)

, 2021
Amyotrophic lateral sclerosis (ALS) is a motor neuron disorder with a terminal outcome, the pathophysiology of which is not yet clearly understood. There are various subtypes of ALS and factors related both to the environment and to genetics which play a role in the development of the condition. This article will give a general overview of ALS and will
openaire   +1 more source