Results 31 to 40 of about 461,814 (280)

Muscle specific kinase (MuSK) activation preserves neuromuscular junctions in the diaphragm but is not sufficient to provide a functional benefit in the SOD1G93A mouse model of ALS

Neurobiology of Disease, 2019
Amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting motor neurons, is characterized by rapid decline of motor function and ultimately respiratory failure.
Arundhati Sengupta-Ghosh, Sara L. Dominguez, Luke Xie, Kai H. Barck, Zhiyu Jiang, Timothy Earr, Jose Imperio, Lilian Phu, Hanna G. Budayeva, Donald S. Kirkpatrick, Hao Cai, Jeffrey Eastham-Anderson, Hai Ngu, Oded Foreman, Maj Hedehus, Michael Reichelt, Isidro Hotzel, Yonglei Shang, Richard A.D. Carano, Gai Ayalon, Amy Easton   +20 more
doaj   +1 more source

Internalization of Muscle-Specific Kinase Is Increased by Agrin and Independent of Kinase-Activity, Lrp4 and Dynamin

Frontiers in Molecular Neuroscience, 2022
Muscle-specific kinase (MuSK) is a receptor tyrosine kinase absolutely required for neuromuscular junction formation. MuSK is activated by binding of motor neuron-derived Agrin to low-density lipoprotein receptor related protein 4 (Lrp4), which forms a ...
Anna Gemza, Cinzia Barresi, Jakob Proemer, Jasmin Hatami, Margarita Lazaridis, Ruth Herbst   +5 more
doaj   +1 more source

Regulatory Function of Sympathetic Innervation on the Endo/Lysosomal Trafficking of Acetylcholine Receptor

Frontiers in Physiology, 2021
Recent studies have demonstrated that neuromuscular junctions are co-innervated by sympathetic neurons. This co-innervation has been shown to be crucial for neuromuscular junction morphology and functional maintenance. To improve our understanding of how
Tatjana Straka, Tatjana Straka, Tatjana Straka, Charlotte Schröder, Andreas Roos, Andreas Roos, Laxmikanth Kollipara, Albert Sickmann, Albert Sickmann, Albert Sickmann, Marion Patrick Ivey Williams, Mathias Hafner, Muzamil Majid Khan, Muzamil Majid Khan, Muzamil Majid Khan, Rüdiger Rudolf, Rüdiger Rudolf, Rüdiger Rudolf   +17 more
doaj   +1 more source

Systematic review of mitochondrial dysfunction and oxidative stress in aging: A focus on neuromuscular junctions

Neural Regeneration Research
Mitochondrial dysfunction and oxidative stress are widely regarded as primary drivers of aging and are associated with several neurodegenerative diseases.
Senlin Chai, Ning Zhang, Can Cui, Zhengyuan Bao, Qianjin Wang, Wujian Lin, Ronald Man Yeung Wong, Sheung Wai Law, Rebecca Schönmehl, Christoph Brochhausen, Wing Hoi Cheung   +10 more
doaj   +1 more source

THE NEUROMUSCULAR JUNCTION DISORDERS [PDF]

Journal of Neurology, Neurosurgery & Psychiatry, 2003
Neuromuscular junction (NMJ) disorders result from destruction, malfunction or absence of one or more key proteins involved in neuromuscular transmission, illustrated diagrammatically in fig 1. The most common pathology is antibody mediated damage or down regulation of ion channels or receptors, resulting in myasthenia gravis (MG), Lambert-Eaton ...
openaire   +2 more sources

Reconstruction of the neuromuscular junction connectome [PDF]

Bioinformatics, 2010
Abstract Motivation: Unraveling the structure and behavior of the brain and central nervous system (CNS) has always been a major goal of neuroscience. Understanding the wiring diagrams of the neuromuscular junction connectomes (full connectivity of nervous system neuronal components) is a starting point for this, as it helps in the study
Ranga Srinivasan, Qing Li 0008, Xiaobo Zhou 0001, Ju Lu, Jeff Lichtman, Stephen T. C. Wong   +5 more
openaire   +5 more sources

Modulation of Microglia by Voluntary Exercise or CSF1R Inhibition Prevents Age-Related Loss of Functional Motor Units

Cell Reports, 2019
Summary: Age-related loss of skeletal muscle innervation by motor neurons leads to impaired neuromuscular function and is a well-established clinical phenomenon. However, the underlying pathogenesis remains unclear. Studying mice, we find that the number
Elisa Giorgetti, Moh Panesar, Yunyu Zhang, Stefanie Joller, Marie Ronco, Michael Obrecht, Christian Lambert, Nathalie Accart, Nicolau Beckmann, Arno Doelemeyer, Ludovic Perrot, Isabelle Fruh, Matthias Mueller, Eliane Pierrel, Serge Summermatter, Michael Bidinosti, Derya R. Shimshek, Sophie Brachat, Mark Nash   +18 more
doaj   +1 more source

Chronobiology of Cancer: How Aging Fuels Oncogenesis at the Molecular Level

Aging and Cancer, EarlyView.
This graphical abstract illustrates the key biological pathways linking aging with cancer development and progression. In the upper left, cumulative exposure to ultraviolet radiation, toxins, and reactive oxygen species (ROS) causes DNA damage and genomic instability, whereas age‐related decline in repair mechanisms, such as ATM/ATR, BER, and NER ...
Anu Singh, Aroonima Misra, Sufian Zaheer
wiley   +1 more source

RNA Sequencing Resolves Cryptic Pathogenic Variants in Mitochondrial Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Mitochondrial diseases are the most common inherited metabolic disorders, characterized by pronounced clinical and genetic heterogeneity that complicates molecular diagnosis. Although DNA‐based sequencing approaches have become standard in genetic testing, up to half of patients remain without a definitive diagnosis.
Zhimei Liu, Xin Duan, Fatemeh Peymani, Jia Wang, Chengjia Bao, Chaolong Xu, Ying Zou, Zixuan Zhang, Yunxi Zhang, Tongyue Li, Martin Pavlov, Junling Wang, Minhan Song, Tianyu Song, Xiaodi Han, Mingxi Sun, Danmin Shen, Ruoyu Duan, Huafang Jiang, Manting Xu, Holger Prokisch, Fang Fang   +21 more
wiley   +1 more source

Unraveling the causes of sarcopenia: Roles of neuromuscular junction impairment and mitochondrial dysfunction

Physiological Reports
Sarcopenia is a systemic skeletal muscle disease characterized by a decline in skeletal muscle mass and function. Originally defined as an age‐associated condition, sarcopenia presently also encompasses muscular atrophy due to various pathological ...
Yanmei Miao, Leiyu Xie, Jiamei Song, Xing Cai, Jinghe Yang, Xinglong Ma, Shaolin Chen, Peng Xie   +7 more
doaj   +1 more source