Article
Multidisciplinary Sciences
Hui -Lam Rachel Kwan, Zora Chui-Kuen Chan, Xinyi Bi, Justyna Kutkowska, Tomasz J. Proszynski, Chi Bun Chan, Chi Wai Lee
Summary: Junctional folds are unique membrane specializations that develop during the postnatal maturation of vertebrate neuromuscular junctions (NMJs). This study demonstrates the progressive development of membrane infoldings via nerve-independent, caveolin-3-dependent mechanisms and identifies their roles in AChR trafficking and redistribution during the structural maturation of NMJs.
Article
Neurosciences
Taro Inoue, Bisei Ohkawara, Samira Bushra, Shunsuke Kanbara, Hiroaki Nakashima, Hiroyuki Koshimizu, Hiroyuki Tomita, Mikako Ito, Akio Masuda, Naoki Ishiguro, Shiro Imagama, Kinji Ohno
Summary: Zonisamide enhances agrin-dependent AChR clustering by upregulating the Nrg1/ErbB signaling pathways at the NMJ.
Article
Biochemistry & Molecular Biology
Nikita Zhilyakov, Arsenii Arkhipov, Artem Malomouzh, Dmitry Samigullin
Summary: Cholinergic neurotransmission plays a key role in both the peripheral and central nervous systems, but certain aspects of its regulation remain unclear. Studies suggest that nicotine can decrease ACh release by enhancing calcium influx through Ca(v)1 channels.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Genetics & Heredity
Kai Ma, Yuhan Chen, Lunni Zhou, Zhizhi Liu, Zhiwei Liu
Summary: Nicotinic acetylcholine receptors (nAChRs) are crucial in regulating the development and function of the nervous system. The study found that the AChR gamma-like gene in Pristella maxillaris could be a subunit of AChRs in muscle, with high expression in embryo and adult tissues.
Review
Biochemistry & Molecular Biology
Kinji Ohno, Bisei Ohkawara, Xin-Ming Shen, Duygu Selcen, Andrew G. Engel
Summary: Congenital myasthenic syndromes (CMS) are a group of heterogeneous disorders characterized by impaired neuromuscular signal transmission due to germline pathogenic variants in genes expressed at the neuromuscular junction (NMJ). A total of 35 genes have been reported in CMS, which can be classified into 14 groups based on the pathomechanical, clinical, and therapeutic features of CMS patients. Diagnosing CMS requires measurement of compound muscle action potentials elicited by repetitive nerve stimulation, and accurate diagnosis always requires genetic studies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Kevin Silva Muller, Felipe Cantore Tiburcio, Jorge Willian Franco de Barros, Cintia Yuri Matsumura, Selma Maria Michelin Matheus
Summary: This study investigated the effects of rosuvastatin and simvastatin exposure during pregnancy on neuromuscular structures in rats. The results showed that these drugs led to alterations in neuromuscular junction morphology, which may be associated with the pathogenesis of statin-associated muscle symptoms (SAMS).
Article
Neurosciences
Ari Dienel, Remya A. Veettil, Kanako Matsumura, H. Alex Choi, Peeyush T. Kumar, Andrey S. Tsvetkov, Jaroslaw Aronowski, Pramod Dash, Spiros L. Blackburn, Devin W. McBride
Summary: Stimulation of alpha 7-AChR promotes neuronal survival and improves neurobehavioral outcomes after SAH, with increased PI3K/Akt activity playing a key role in mediating these effects. Knockout studies and cell culture experiments support a direct effect of alpha 7-AChR agonism on promoting cell survival.
EXPERIMENTAL NEUROLOGY
(2021)
Article
Developmental Biology
Sara Bsharat, Emanuela Monni, Tania Singh, Jenny K. Johansson, Kavya Achanta, Ludivine Bertonnier-Brouty, Anja Schmidt-Christensen, Dan Holmberg, Zaal Kokaia, Rashmi B. Prasad, Isabella Artner
Summary: Hormone secretion from pancreatic islets is essential for glucose homeostasis, and loss or dysfunction of islet cells is a hallmark of type 2 diabetes. Maf transcription factors are crucial for establishing and maintaining adult endocrine cell function. Here, we report that MafB deficiency impairs β cell clustering and islet formation, but also coincides with loss of neurotransmitter and axon guidance receptor gene expression.
Review
Biochemistry & Molecular Biology
Ellya Bukharaeva, Venera Khuzakhmetova, Svetlana Dmitrieva, Andrei Tsentsevitsky
Summary: Adrenoceptor activators and blockers are commonly used in the clinical treatment of cardiovascular, pulmonary, and neurodegenerative diseases by regulating neurotransmission at the neuromuscular junction. Understanding the molecular mechanisms of these drugs may lead to the development of highly effective and well-tolerated sympathomimetics for treating various neurodegenerative diseases with synaptic defects.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Engineering, Biomedical
Ji Hyun Kim, In Kap Ko, Myung Jae Jeon, Ickhee Kim, Margaret Maria Vanschaayk, Anthony Atala, James J. Yoo
Summary: Efficient innervation is crucial for successful grafting and functional recovery of bioengineered skeletal muscle constructs. In this study, a 3D bioprinted human skeletal muscle construct with pre-formed AChR clusters is developed and shown to accelerate innervation in vivo, leading to functional muscle reconstruction in a rat model of pelvic floor muscle injury.
ACTA BIOMATERIALIA
(2021)
Article
Geriatrics & Gerontology
Li-Kai Tsai, I-Shin Chen, Chi-Chao Chao, Hsueh-Wen Hsueh, Hsin-Hsiung Chen, Yun-Hsin Huang, Rong-Wei Weng, Tzu-Yun Lai, Yi-Chieh Tsai, Yeou-Ping Tsao, Show-Li Chen
Summary: The study revealed the important role of NRIP in the neuromuscular junction, showing its co-localization and interaction with AChR-associated proteins. Anti-NRIP autoantibody was found in patients with myasthenia gravis, potentially exacerbating disease severity.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2021)
Review
Biochemistry & Molecular Biology
Bisei Ohkawara, Mikako Ito, Kinji Ohno
Summary: This review focuses on secreted signaling molecules that regulate the clustering of acetylcholine receptors (AChRs) at the neuromuscular junction (NMJ), including neural agrin, Lrp4, MuSK, Rspo2, Fgf18, and Ctgf. These molecules play crucial roles in ensuring efficient signal transduction at the NMJ.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biology
Marta Morotti, Alessandro Gaeta, Cristina Limatola, Myriam Catalano, Maria Amalia Di Castro, Francesca Grassi
Summary: This study found that the absence of dystrophin does not affect the distribution of AChRs at the endplate, but the formation and disassembly of extra-synaptic AChR clusters may be regulated differently in mdx mice.
Article
Biochemistry & Molecular Biology
Jun Yang, Jun Cao, Su Min, Ping Li, Feng Lv, Li Ren
Summary: Immobilization-induced Neuromuscular Dysfunction (NMD) significantly impacts the wellbeing of patients in Intensive Care Units, and the underlying mechanism of NMD is still not well understood. In this study, an immobilization rat model was used to investigate the role of NRG-1/ErbB pathway in NMD. The results showed that exogenous recombinant human neuregulin-1 (rhNRG-1) alleviated muscle loss, enhanced e-nAChR expression, and suppressed γ-and α7-nAChR production, suggesting that rhNRG-1 could be a promising therapeutic option for immobilization-induced myopathy.
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS
(2023)
Article
Biochemistry & Molecular Biology
Paloma Alvarez-Suarez, Natalia Nowak, Anna Protasiuk-Filipunas, Hiroyuki Yamazaki, Tomasz J. Proszyriski, Marta Gawor
Summary: The study reveals the role of drebrin in the postsynaptic machinery at NMJs, including its colocalization with AChRs and enrichment at synaptic podosomes, as well as interactions with rapsyn and EB3.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)