Article
Cell Biology
Jakob Proemer, Cinzia Barresi, Ruth Herbst
Summary: Muscle-specific kinase (MuSK) is the key regulator of neuromuscular junction development and its activation and signaling are tightly regulated. Recent studies using omics techniques have contributed to a better understanding of MuSK signaling. Impaired MuSK signaling causes muscle weakness, but the underlying pathophysiology is often unclear. This review focuses on recent advances in deciphering MuSK activation and downstream signaling, as well as the role of MuSK in non-muscle tissue.
CELLULAR SIGNALLING
(2023)
Article
Neurosciences
Anselmo Canciani, Cristina Capitanio, Serena Stanga, Silvia Faravelli, Luigi Scietti, Lisa Mapelli, Teresa Soda, Egidio D'Angelo, Pascal Kienlen-Campard, Federico Forneris
Summary: Neurotrypsin is a specific nervous system serine protease with important roles in synaptic plasticity and neuromuscular junction degeneration. This study provides insights into the regulatory effects of NT on agrin signaling, as well as the molecular mechanisms and biological significance of NT-agrin interactions. The results have important implications for understanding the pathology of neurological disorders.
MOLECULAR NEUROBIOLOGY
(2022)
Article
Neurosciences
Kiel G. Ormerod, Anthony E. Scibelli, J. Troy Littleton
Summary: The Drosophila neuromuscular system has been used to study synaptic development and function, but little is known about specific synaptic alterations on neuromuscular transduction and muscle contractility. Studies show that larval muscle contraction force increases with motoneuron stimulation frequency and duration, with plasticity between 5 and 40 Hz. Genetic and pharmacological manipulations of Ca2+ regulation components affect muscle contraction strength and timing. Identification of a FMRFa peptide and its signaling pathway dramatically improves muscle performance, showcasing Drosophila as a model for studying excitation-contraction coupling.
JOURNAL OF PHYSIOLOGY-LONDON
(2022)
Article
Cell Biology
Jing Zhao, Linlin Yin, Lin Jiang, Li Hou, Ling He, Chunyan Zhang
Summary: The study revealed the essential role of PTEN nuclear translocation in neuronal injury following oxygen-glucose deprivation in rat cortical neurons. The mechanism may involve modulation of cell signaling pathways and apoptosis-related proteins. Preventing PTEN nuclear translocation could be a potential strategy for preventing brain injury following hypoxia-ischemia.
Review
Neurosciences
Eleanor J. Jones, Shin-Yi Chiou, Philip J. Atherton, Bethan E. Phillips, Mathew Piasecki
Summary: A motor unit (MU) consists of a neuron cell body, its axon, and the muscle fibers it controls. Age-related reductions in the number of MUs are observed, but MUs can undergo remodeling to rescue denervated muscle fibers. This remodeling process involves axonal sprouting and the formation of structures around the neuromuscular junction, relying on neurotrophins and coordinated signaling. While exercise in older age has shown benefits for skeletal muscle, research in humans on MU remodeling is limited.
JOURNAL OF PHYSIOLOGY-LONDON
(2022)
Review
Biochemistry & Molecular Biology
Adel E. E. Khairullin, Sergey N. N. Grishin, Ayrat U. U. Ziganshin
Summary: The purine signaling system, represented by purine and pyrimidine nucleotides and nucleosides, plays a role through the adenosine, P2X and P2Y receptor families. While P2 receptors have a minor role in physiological conditions, they become more significant in certain pathophysiological conditions, functioning as dominant signaling molecules. The diversity and distribution of P2 receptors make them an attractive target for new mechanism drugs.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Developmental Biology
Jun Yu, Marilyn Janice Oentaryo, Chi Wai Lee
Summary: The study demonstrates the essential role of local protein synthesis in presynaptic differentiation at developing neuromuscular junctions. Newly synthesized proteins play a crucial role at agrin bead-neurite contacts. Axonal MT1-MMP translation is shown to be vital in mediating agrin-induced presynaptic differentiation, in addition to the recognized long-range transport of MT1-MMP proteins.
Article
Medicine, Research & Experimental
Lea Lescouzeres, Cedric Hassen-Khodja, Anais Baudot, Benoit Bordignon, Pascale Bomont
Summary: Giant axonal neuropathy (GAN) is a fatal neurodegenerative disorder without effective treatment. Using the gan zebrafish model, researchers conducted a pharmacological screening and identified five drugs that restored locomotion and axonal outgrowth in GAN. The results provide potential drug candidates for the treatment of GAN and may also benefit other neuromuscular diseases.
EMBO MOLECULAR MEDICINE
(2023)
Article
Neurosciences
Madeleine E. Cunningham, Rhona McGonigal, Jennifer A. Barrie, Denggao Yao, Hugh J. Willison
Summary: This study is the first investigation of axonal calcium dynamics in a mouse model of Guillain-Barre acute accent syndrome, demonstrating the proximal reach of calcium influx following an injury which is confined to the most distal parts of the motor axon. Additionally, it is shown that calpain inhibition remains a promising candidate for both acute and sub-acute consequences of calcium-induced calpain activation.
EXPERIMENTAL NEUROLOGY
(2022)
Article
Neurosciences
Emily L. Hendricks, Ireland R. Smith, Bruna Prates, Fatemeh Barmaleki, Faith L. W. Liebl
Summary: The study reveals the role of CD63 and its homologs, Tsp42Ee and Tsp42Eg, in regulating synaptic vesicle pools and synaptic dysfunction. These proteins negatively regulate endocytosis and positively regulate neurotransmitter release. They also influence the synaptic cytoskeleton, membrane composition, and the localization of vesicle-associated proteins.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Article
Cell Biology
S. L. Morris, S. T. Brady
Summary: Tau, a phosphoprotein found in neurons, plays a major role in regulating fast axonal transport (FAT) through activation of neuronal signaling pathways. The exposure of tau PAD is an early event in tauopathies and is a major contributing factor to neurodegeneration. These findings suggest that the transient activation of the PP1-GSK3 β signaling pathway through locally regulated PAD exposure is crucial for cargo delivery and neurite outgrowth in developing neurons.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2023)
Article
Neurosciences
Matthew J. J. Fogarty, Obaid U. U. Khurram, Carlos B. B. Mantilla, Gary C. C. Sieck
Summary: The study suggests that chronic TrkB kinase inhibition in TrkB(F616) rats results in a significant worsening of diaphragm neuromuscular transmission, which acute BDNF treatment cannot rescue. Additionally, chronic TrkB kinase inhibition does not affect the apposition of pre-synaptic terminals and post-synaptic endplates at diaphragm NMJs.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Review
Biochemistry & Molecular Biology
Friederike Cuello, Friedrich W. Herberg, Konstantina Stathopoulou, Philipp Henning, Simon Diering
Summary: Pathologies such as cancer, inflammatory and cardiac diseases are often associated with long-term oxidative stress, leading to protein dysfunction and failed trials of systemic antioxidant therapy. In cardiac myocytes, cardiac contractility is mainly driven by the beta-adrenoceptor-signaling cascade activating PKA-mediated phosphorylation for positive inotropic and lusitropic effects. Both kinase and phosphatase are redox-sensitive and play crucial roles in regulating cardiac contractile function.
Article
Biology
Yan Tang, Haihong Zong, Hyokjoon Kwon, Yunping Qiu, Jacob B. Pessin, Licheng Wu, Katherine A. Buddo, Ilya Boykov, Cameron A. Schmidt, Chien-Te Lin, P. Darrell Neufer, Gary J. Schwartz, Irwin J. Kurland, Jeffrey E. Pessin
Summary: Cholinergic and sympathetic counter-regulatory networks regulate various physiological functions through increased glycolysis, including blood pressure, heart rate, and energy balance. Increased cholinergic signaling has metabolic benefits, particularly in increasing energy expenditure and heat production during cold exposure.
Article
Biochemistry & Molecular Biology
Marcin Pezinski, Kamila Maliszewska-Olejniczak, Patrycja Daszczuk, Paula Mazurek, Pawel Niewiadomski, Maria Jolanta Redowicz
Summary: Tks5 is identified as a crucial regulator of the postsynaptic machinery, involved in remodeling synaptic podosomes to maintain AChR clusters and regulate their turnover. This study provides the first evidence of Tks5's key role in synaptic machinery regulation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Multidisciplinary Sciences
Xin Sun, Pan P. Li, Shanshan Zhu, Rachael Cohen, Leonard O. Marque, Christopher A. Ross, Stefan M. Pulst, Ho Yin Edwin Chan, Russell L. Margolis, Dobrila D. Rudnicki
SCIENTIFIC REPORTS
(2015)
Article
Clinical Neurology
Pan P. Li, Xin Sun, Guangbin Xia, Nicolas Arbez, Sharan Paul, Shanshan Zhu, H. Benjamin Peng, Christopher A. Ross, Arnulf H. Koeppen, Russell L. Margolis, Stefan M. Pulst, Tetsuo Ashizawa, Dobrila D. Rudnicki
ANNALS OF NEUROLOGY
(2016)
Article
Cell Biology
Qian Zhang, Ho Tsoi, Shaohong Peng, Pan P. Li, Kwok-Fai Lau, Dobrila D. Rudnicki, Jacky Chi-Ki Ngo, Ho Yin Edwin Chan
DISEASE MODELS & MECHANISMS
(2016)
Article
Anatomy & Morphology
Pan P. Li, Raghavan Madhavan, H. Benjamin Peng
DEVELOPMENTAL DYNAMICS
(2012)
Article
Biochemistry & Molecular Biology
Xin Sun, Leonard O. Marque, Zachary Cordner, Jennifer L. Pruitt, Manik Bhat, Pan P. Li, Geetha Kannan, Ellen E. Ladenheim, Timothy H. Moran, Russell L. Margolis, Dobrila D. Rudnicki
HUMAN MOLECULAR GENETICS
(2014)
Article
Cell Biology
Pan P. Li, Cheng Chen, Chi-Wai Lee, Raghavan Madhavan, H. Benjamin Peng
MOLECULAR BIOLOGY OF THE CELL
(2011)
Article
Cell Biology
Cheng Chen, Pan P. Li, Raghavan Madhavan, H. Benjamin Peng
MOLECULAR BIOLOGY OF THE CELL
(2012)
Article
Multidisciplinary Sciences
Pan P. Li, Jie J. Zhou, Min Meng, Raghavan Madhavan, H. Benjamin Peng
Article
Clinical Neurology
Pan P. Li, Roumita Moulick, Hongxuan Feng, Xin Sun, Nicolas Arbez, Jing Jin, Leonard O. Marque, Erin Hedglen, H. Y. Edwin Chan, Christopher A. Ross, Stefan M. Pulst, Russell L. Margolis, Sarah Woodson, Dobrila D. Rudnicki
Summary: The study demonstrates that expATXN2 RNA induces neuronal cell death and interacts abnormally with RNA binding proteins involved in RNA metabolism. The findings suggest a point of convergence in the pathogeneses of repeat expansion diseases, with potential therapeutic implications.
MOVEMENT DISORDERS
(2021)