Results 141 to 150 of about 308,172 (351)

Allosteric Modulation of Pathological Ataxin‐3 Aggregation: A Path to Spinocerebellar Ataxia Type‐3 Therapies

Advanced Science, EarlyView.
This study uncovers a new allosteric site in the Josephin domain of ataxin‐3 targeted by the molecular tweezer CLR01, which modulates protein aggregation, improves synaptic function in neuronal cells, and delays motor dysfunction in animal models.
Alexandra Silva, Sara Duarte‐Silva, Pedro M. Martins, Beatriz Rodrigues, Débora Serrenho, Daniela Vilasboas‐Campos, Andreia Teixeira‐Castro, Julio Vieyto‐Nuñez, Joel Mieres‐Perez, Francisco Figueiredo, Martyna Podlasiak, Tatiana Van‐der‐Kellen, Joana Fraga, James Noble, Carter Lantz, Niki Sepanj, Daniela Monteiro‐Fernandes, Sara Guerreiro, Andreia Neves‐Carvalho, Joana Pereira‐Sousa, Frank‐Gerrit Klärner, Thomas Schrader, Joseph A. Loo, Annalisa Pastore, Elsa Sanchez‐Garcia, Gal Bitan, Ana Luísa Carvalho, Patrícia Maciel, Sandra Macedo‐Ribeiro   +28 more
wiley   +1 more source

Equine degenerative myeloencephalopathy: prevalence, impact, and management. [PDF]

, 2018
Equine neuroaxonal dystrophy/equine degenerative myeloencephalopathy is an inherited neurodegenerative disorder affecting many horse breeds. Clinical signs include a symmetric ataxia and an abnormal stance at rest, similar to cervical vertebral ...
Burns, Erin N, Finno, Carrie J
core  

NAD⁺ Reduction in Glutamatergic Neurons Induces Lipid Catabolism and Neuroinflammation in the Brain via SARM1

Advanced Science, EarlyView.
NAD⁺ homeostasis maintains neuronal integrity through opposing actions of NMNAT2 and SARM1. Loss of NMNAT2 in glutamatergic neurons reprograms cortical metabolism from glucose to lipid catabolism, depletes lipid stores, and triggers inflammation and neurodegeneration.
Zhen‐Xian Niou, Sen Yang, Andrea Enriquez, Nino A. Espinas, Anoosha Sri, Caliel D. Hines, Jason M. Tennessen, Chia‐Shan Wu, Jui‐Yen Huang, Hui‐Chen Lu   +9 more
wiley   +1 more source

A Refined Vestibular Romberg Test to Differentiate Somatosensory from Vestibular-Induced Disequilibrium

Diagnostics
Background: The vestibular Romberg test, which assesses the deterioration of balance while standing on rubber foam with closed eyes, is a well-established method in the physical neurological assessment of patients with peripheral vestibulopathy.
Evangelos Anagnostou, Anastasia Gamvroula, Maria Kouvli, Evangelia Karagianni, George Stranjalis, Maria Skoularidou, Theodosis Kalamatianos   +6 more
doaj   +1 more source

TMEM131‐Mediated Soluble TRAIL Triggered Type II Alveolar Epithelial Cell Senescence in Radiation‐Induced Lung Injury

Advanced Science, EarlyView.
TMEM131 recruits the COPII complex to accelerate TRAIL transportation from endoplasmic reticulum to Golgi apparatus, and promotes soluble TRAIL secretion. TRAIL inhibits mitophagy and induces senescence through DR5 receptor in type II alveolar epithelial cells, ultimately driving radiation‐induced lung injury (RILI) progression.
Linzhi Han, Chunsheng Wang, Xiuli Guo, Yuan Luo, Jianguo Zhang, Fajian He, Zihang Zeng, Jiang Luo, Wen Ouyang, Yan Gong, Conghua Xie   +10 more
wiley   +1 more source

Unilateral Oculomotor Nerve Palsy Following Campylobacter Infection: A Mild Form of Miller Fisher Syndrome without Ataxia

, 2017
Tatsuya Ueno, Tomoya Kon, Aiichiro Kurihara, Masahiko Tomiyama   +3 more
openalex   +2 more sources

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia [PDF]

, 2002
ANA HAUSSE, Y Aggoun, D Bonnet, D Sidi, A Munnich, A Rötig, P Rustin   +6 more
openalex   +1 more source

Ataxia telangiectasia

Seminars in Pediatric Neurology, 1998
The cloning of ATM in 1995, the gene responsible for ataxia-telangiectasia, opened a dimension of biological research that is as complex and intriguing to cell biologists as this classic disorder has been to clinicians for four decades. The phenotype is both variable and stereotyped, with significant differences between patients in the rate of ...
openaire   +2 more sources

KRAS Withdrawal in Cholangiocarcinoma Leads to Immune Infiltration and Tumor Regression

Advanced Science, EarlyView.
Cholangiocarcinoma (CCA) driven by oncogenic KRAS depends on its continuous activation for tumor maintenance. Using a conditional KRAS model, the authors show that turning off KRAS triggers rapid tumor regression accompanied by immune cell infiltration and cytokine release. The findings uncover a KRAS–senescence–immune signaling axis and highlight KRAS
Youwei Qiao, Matthew F. Yee, Chaitanya N. Parikh, Boyang Ma, Yueying Cao, Yu‐Huan Shih, Joae Qiong Wu, Marcus Ruscetti, Shun‐Qing Liang, Wen Xue   +9 more
wiley   +1 more source

Impact of ataxia aetiology on self-reported mental health, fatigue, cognition and ataxia symptom severity

Discover Psychology
Introduction It has been increasingly recognised that the impact of Ataxia extends beyond physical and motor symptomology. However, it is less known whether self-reported non-motor and ataxia symptom severity varies across ataxias of differing aetiology,
Emma Jenkins, Warren Dunger
doaj   +1 more source