Article
Clinical Neurology
Guillem Modol-Caballero, Belen Garcia-Lareu, Mireia Herrando-Grabulosa, Sergi Verdes, Ruben Lopez-Vales, Gemma Pages, Miguel Chillon, Xavier Navarro, Assumpcio Bosch
Summary: Targeted expression of GDNF in muscle can delay disease progression in a mouse model of ALS by protecting neuromuscular function, increasing innervated neuromuscular junctions, preserving spinal motoneurons, and reducing glial reactivity. These beneficial effects are attributed to a paracrine protective mechanism from the muscle to the motoneurons mediated by GDNF, with no adverse secondary effects detected, highlighting the potential for ALS therapy.
Article
Biochemistry & Molecular Biology
Guzel F. Zakyrjanova, Andrei N. Tsentsevitsky, Eva A. Kuznetsova, Alexey M. Petrov
Summary: Inflammatory reactions can induce changes in the neuromuscular system. The oxysterol 25-hydroxycholesterol (25HC) may regulate neurotransmission and contribute to neuromuscular activity via activation of lipid raft-associated LXRs. 25HC-mediated increase in ROS production may modulate signaling pathways involving estrogen receptor alpha - Gi-protein - G beta gamma - phospholipase C - Ca2+ - protein kinase C.
FREE RADICAL BIOLOGY AND MEDICINE
(2021)
Review
Neurosciences
Sarah Lepine, Maria Jose Castellanos-Montiel, Thomas Martin Durcan
Summary: The abnormal synaptic function of TDP-43 is closely related to NMJ disruption in ALS, and it may exert its effects by influencing molecular mechanisms within motor neurons, skeletal muscles, and glial cells.
TRANSLATIONAL NEURODEGENERATION
(2022)
Article
Neurosciences
Sagar Verma, Shiffali Khurana, Abhishek Vats, Bandana Sahu, Nirmal Kumar Ganguly, Pradip Chakraborti, Mandaville Gourie-Devi, Vibha Taneja
Summary: Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by degeneration of motor neurons and potential early pathology at the neuromuscular junction (NMJ). However, the events triggering NMJ disassembly in ALS remain unclear.
MOLECULAR NEUROBIOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Jianxun Yi, Ang Li, Xuejun Li, Kiho Park, Xinyu Zhou, Frank Yi, Yajuan Xiao, Dosuk Yoon, Tao Tan, Lyle W. Ostrow, Jianjie Ma, Jingsong Zhou
Summary: Respiratory failure due to progressive respiratory muscle weakness is the most common cause of death in amyotrophic lateral sclerosis (ALS). Research has shown that the muscle protein MG53 can repair NMJ damage, protect the diaphragm muscle from injury, preserve NMJ integrity, and slow the progression of ALS. This indicates MG53 may be a potentially safe and effective therapy for ALS.
Article
Clinical Neurology
Claudia Santos Silva, Marta Gromicho, Miguel Oliveira Santos, Susana Pinto, Michael Swash, Mamede de Carvalho
Summary: In this study, the prevalence of thyroid dysfunction in amyotrophic lateral sclerosis (ALS) patients was investigated. The results showed that ALS patients had a lower prevalence of hypothyroidism, hyperthyroidism, and overall thyroid dysfunction compared to controls, but similar to the national epidemiological data for thyroid disease. This study is consistent with previous findings and suggests that thyroid dysfunction is not associated with ALS.
NEUROLOGICAL SCIENCES
(2022)
Article
Zoology
Wen-Yo Tu, Wentao Xu, Jianmin Zhang, Shuyuan Qi, Lei Bai, Chengyong Shen, Kejing Zhang
Summary: Amyotrophic lateral sclerosis (ALS) is a devastating motoneuron disease characterized by the loss of control of skeletal muscles. Degeneration of neuromuscular junctions (NMJs) is an early event in ALS. Poly-GA, the most abundant form of DPR in C9-ALS, is released from cells and impairs NMJs through inhibiting Agrin-MuSK signaling.
ZOOLOGICAL RESEARCH
(2023)
Article
Geriatrics & Gerontology
Sunghee Kim, Subin An, Jinwoo Lee, Yideul Jeong, Chang-Lim You, Hyebeen Kim, Ju-Hyeon Bae, Chae-Eun Yun, Dongryul Ryu, Gyu-Un Bae, Jong-Sun Kang
Summary: The cell adhesion molecule Cdon plays a critical role in motor neuron function and nerve repair. Mice lacking Cdon exhibit middle age onset lethality and aging-related decline in motor function. Additionally, these mice also show impairment in motor function recovery and incomplete neuromuscular junction occupation.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2023)
Article
Neurosciences
Pablo Rozas, Cristina Pinto, Francisca Martinez Traub, Rodrigo Diaz, Viviana Perez, Daniela Becerra, Patricia Ojeda, Jorge Ojeda, Madison T. Wright, Jessica Mella, Lars Plate, Juan Pablo Henriquez, Claudio Hetz, Danilo B. Medinas
Summary: Overexpression of ERp57 in experimental ALS using mutant SOD1 mice delayed deterioration of electrophysiological activity and maintained muscle innervation at early-symptomatic stage, improving motor performance without affecting survival. The neuroprotective effects of ERp57 overexpression were correlated with increased levels of synaptic and actin cytoskeleton proteins in the spinal cord.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)
Review
Cell Biology
Xin Jiang, Yingjun Guan, Zhenhan Zhao, Fandi Meng, Xuemei Wang, Xueshuai Gao, Jinmeng Liu, Yanchun Chen, Fenghua Zhou, Shuanhu Zhou, Xin Wang
Summary: The WNT signaling pathway is crucial in the pathophysiology of ALS and may serve as a potential therapeutic target. Pathways associated with neurodegeneration and inflammation are key in ALS therapy.
Review
Behavioral Sciences
Sanjeev Kharel, Rajeev Ojha, Veeramani Preethish-Kumar, Riwaj Bhagat
Summary: This study investigated the role of C-reactive protein (CRP) in amyotrophic lateral sclerosis (ALS). The results showed a significant increase in CRP levels among ALS patients compared to healthy controls in most of the studies. In addition, CRP levels were found to be correlated with ALS functional rating scale and disease progression. However, more large-scale studies are needed to evaluate the value and accuracy of CRP as a prognostic biomarker in ALS, taking into account confounding factors.
BRAIN AND BEHAVIOR
(2022)
Article
Biochemistry & Molecular Biology
Irina G. Bryndina, Maria N. Shalagina, Vladimir A. Protopopov, Alexey V. Sekunov, Andrey L. Zefirov, Guzalia F. Zakirjanova, Alexey M. Petrov
Summary: The study suggests that denervation can increase lipid raft formation and attenuate muscle disuse-induced changes, possibly due to the decrease in Cer levels in the raft fraction. It also highlights the importance of motor and afferent inputs in controlling Cer distribution and the stability of lipid rafts in muscle membranes.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Clinical Neurology
Doaa M. Taha, Benjamin E. Clarke, Claire E. Hall, Giulia E. Tyzack, Oliver J. Ziff, Linda Greensmith, Bernadett Kalmar, Mhoriam Ahmed, Aftab Alam, Eric P. Thelin, Nuria Marco Garcia, Adel Helmy, Christopher R. Sibley, Rickie Patani
Summary: This study investigates the cellular autonomy and uniformity of astrocyte reactive transformation in different genetic forms of amyotrophic lateral sclerosis. By using enriched and human induced pluripotent stem cell-derived astrocytes from patients with VCP and SOD1 mutations, the study shows that reactive transformation can occur cell-autonomously in ALS astrocytes and there is molecular and functional heterogeneity between different disease-causing mutations.
Article
Clinical Neurology
Philippe Corcia, Pascal Lejeune, Patrick Vourc'h, Stephane Beltran, Anne-Sophie Piegay, Helene Blasco, Vincent Meininger
Summary: This study characterized the prototypical phenotype of patients with amyotrophic lateral sclerosis (ALS) associated with PFN1 mutations and identified clinical indications for testing mutations in this gene. The main clinical findings for familial ALS linked to PFN1 were identified as pedigrees with over five cases, an onset age around 50 years, onset in the lower limbs, and the absence of cognitive impairment. The similarities with other ALS mutations prompt a review of ALS classifications based on both phenotype and genotype.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Carolyn Tallon, Anjali Sharma, Zhi Zhang, Ajit G. Thomas, Justin Ng, Xiaolei Zhu, Amanda Donoghue, Michael Schulte, Tawnjerae R. Joe, Siva P. Kambhampati, Rishi Sharma, Kevin Liaw, Sujatha Kannan, Rangaramanujam M. Kannan, Barbara S. Slusher
Summary: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by muscle weakness and neuromuscular junction denervation. Glutamate has been identified as an important neurotransmitter for NMJ development and maintenance. Inhibiting elevated GCPII activity in SOD1(G93A) muscle can prolong muscle function and delay NMJ denervation in ALS.
Article
Cell Biology
Chulpan R. Gafurova, Andrei N. Tsentsevitsky, Alexey M. Petrov
Summary: The study revealed that different types of synaptic vesicles (SVs) in nerve terminals exhibit distinct behaviors in response to low- and high-frequency stimulations. At least two SV pools were found to operate during prolonged stimulation, one for maintaining release at different frequencies and the other for responding to increasing activity.
CELLULAR AND MOLECULAR NEUROBIOLOGY
(2022)
Article
Cell Biology
A. N. Tsentsevitsky, A. M. Petrov
Summary: L-type Ca2+ channels play a key role in regulating neurotransmitter release at neuromuscular junctions. They can enhance and desynchronize neurotransmitter release, and are regulated by the P2Y-R/PKC axis in frogs. Additionally, the effects of ATP on neurotransmitter release are dependent on LTCC function and differ between distal and proximal parts of the NMJ.
CELLULAR AND MOLECULAR NEUROBIOLOGY
(2022)
Article
Medicine, Research & Experimental
Alexey M. Petrov, Guzalia F. Zakirjanova, Irina Kovyazina, Andrei N. Tsentsevitsky, Ellya A. Bukharaeva
Summary: This study investigated the influence of nerve activity and adrenoceptor stimulation on exocytosis modes in the motor nerve terminal. It was found that high frequency activity promoted neurotransmitter release via kiss-and-run mechanism, while noradrenaline and alpha 2-adrenoreceptors agonist favored full-collapse exocytosis. This has implications for the regulation of respiratory system during stress.
Article
Neurosciences
Andrei N. Tsentsevitsky, Eduard F. Khaziev, Irina V. Kovyazina, Alexey M. Petrov
Summary: The study found that GIRK channels can regulate the timing of neurotransmitter release and act as a positive modulator of synaptic vesicle exocytosis in frog motor nerve terminals, partially through L-type calcium channels. Additionally, GIRK channels are key players in the feedback control of neurotransmitter release by muscarinic acetylcholine receptors.
Review
Biochemistry & Molecular Biology
Ulia G. Odnoshivkina, Eva A. Kuznetsova, Alexey M. Petrov
Summary: Cholesterol is an important component of the plasma membrane and serves as a precursor for biologically active compounds. 25-hydroxycholesterol (25-HC) plays a crucial role in regulating cellular cholesterol homeostasis and has been found to be upregulated in various neurological disorders and inflammation. Its molecular targets include transcription factors, G protein-coupled receptors, ion channels, and adhesive molecules. The diverse binding proteins of 25-HC indicate its ability to affect multiple physiological and pathological processes. This review focuses on the production and universal role of 25-HC in controlling cellular cholesterol homeostasis, as well as its effects as a signaling molecule in neuroinflammation and synaptic transmission.
BIOCHEMISTRY-MOSCOW
(2022)
Review
Biochemistry & Molecular Biology
Ellya A. Bukharaeva, Andrey Skorinkin, Dmitry Samigullin, Alexey M. Petrov
Summary: Effective transmission of excitation in neuromuscular junctions requires a certain level of depolarization to trigger action potential spreading. The amplitude of end-plate potential, which depends on the synchronization and release rate of acetylcholine quanta, is influenced by presynaptic autoreceptors. The timing characteristics of neurotransmitter release play a crucial role in the plasticity and efficacy of synaptic transmission.
Article
Biochemistry & Molecular Biology
Marat A. Mukhamedyarov, Aydar N. Khabibrakhmanov, Venera F. Khuzakhmetova, Arthur R. Giniatullin, Guzalia F. Zakirjanova, Nikita V. Zhilyakov, Kamilla A. Mukhutdinova, Dmitry V. Samigullin, Pavel N. Grigoryev, Andrey V. Zakharov, Andrey L. Zefirov, Alexey M. Petrov
Summary: Amyotrophic lateral sclerosis (ALS) is a disease characterized by skeletal muscle denervation, motor neuron loss, and respiratory failure. Mutations in the FUS gene are a common genetic cause of ALS, resulting in degeneration. In mutant FUS mice at the pre-onset stage, early structural and functional alterations in diaphragm neuromuscular junctions (NMJs) were observed. Lipid peroxidation and decreased lipid raft staining were found in the mutant mice. Despite preserved end-plate structure, increased levels of presynaptic proteins and impaired neurotransmitter release were observed in FUS mice. These findings suggest that alterations in membrane properties, synapsin 1 levels, and calcium kinetics could be early indicators of nascent NMJ pathology in ALS.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Medicine, Research & Experimental
Andrei N. Tsentsevitsky, Chulpan R. Gafurova, Kamilla A. Mukhutdinova, Arthur R. Giniatullin, Nikita S. Fedorov, Artem I. Malomouzh, Alexey M. Petrov
Summary: In this study on the diaphragm neuromuscular junctions of mice, it was found that the release of neurotransmitters is regulated by secretory sphingomyelinases (SMases), which enhance mobilization of synaptic vesicles and facilitate full fusion mode of exocytosis while suppressing neurotransmitter transmission when acting on vesicular membranes.
Article
Biochemistry & Molecular Biology
Guzalia F. Zakirjanova, Arthur R. Giniatullin, Chulpan R. Gafurova, Artem I. Malomouzh, Nikita S. Fedorov, Arthur N. Khaziev, Andrei N. Tsentsevitsky, Alexey M. Petrov
Summary: This study investigates the effects of enzymatic cholesterol oxidation on neuromuscular transmission in mice diaphragms. The results show that cholesterol oxidation disrupts lipid-ordering in synaptic membranes, decreases synaptic delay and exocytosis probability, enhances neurotransmitter and dye release, and inhibits extracellular acetylcholine accumulation. Additionally, cholesterol oxidation affects the mode of exocytosis and may be related to lipid raft disruption.
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS
(2023)
Article
Biochemistry & Molecular Biology
Julia G. Odnoshivkina, Guzel V. Sibgatullina, Alexey M. Petrov
Summary: This study discovers that neurotransmitter release from sympathetic terminals plays a crucial role in heart regulation. Monitoring presynaptic exocytotic activity in mice atrial tissue, it is found that high [K+]o depolarization causes the release of FFN511, a false fluorescent neurotransmitter, and this release is augmented by the neurotransmitter uptake inhibitor reserpine. In conclusion, neurotransmitter release is essential for heart regulation, and can be influenced by factors such as depolarization and neurotransmitter uptake inhibitors.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES
(2023)
Article
Biochemistry & Molecular Biology
Andrei N. Tsentsevitsky, Venera F. Khuzakhmetova, Ellya A. Bukharaeva, Alexey M. Petrov
Summary: This study found that alpha 2-adrenoreceptors (ARs) are important regulators of neuromuscular transmission through G protein-gated inwardly rectifying potassium (GIRK) channels, and synaptic vesicle exocytosis depends on calcium influx through voltage-gated calcium channels (VGCCs).
NEUROCHEMICAL RESEARCH
(2023)
Article
Geosciences, Multidisciplinary
N. G. Razzhigaeva, T. A. Kopoteva, T. R. Makarova, T. A. Grebennikova, M. S. Lyashchevskaya, L. M. Mokhova, L. A. Ganzey, E. P. Kudryavtseva, A. M. Panichev, M. A. Klimin, Kh. A. Arslanov, F. E. Maksimov, A. Yu. Petrov
Summary: A comprehensive study of the Bolshaya Ussurka River basin revealed climate changes over the past 2900 years, including periods of drought and humidity fluctuations, as well as the impact of environmental changes on vegetation. The study also found that human intervention has led to an increase in the scale of floods.
DOKLADY EARTH SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
U. G. Odnoshivkina, A. M. Petrov
Summary: During inflammation, the conversion of cholesterol to 25-hydroxycholesterol (25HC) increases in macrophages, leading to the activation of liver X receptors (LXRs) which regulate cardiac hypertrophy and metabolism. 25HC affects atrial contractions and inhibits the response to beta-adrenoceptor stimulation. The effects of 25HC are dependent on LXR receptors and lipid rafts.
JOURNAL OF EVOLUTIONARY BIOCHEMISTRY AND PHYSIOLOGY
(2022)
Proceedings Paper
Computer Science, Artificial Intelligence
Olga N. Masina, Olga V. Druzhinina, Alexey A. Petrov
Summary: This paper focuses on the synthesis and analysis of a controlled model of a belt conveyor with switching modes, taking into account axial resistance and dynamic changes in the angle between the horizontal plane and the conveyor belt. It discusses the construction of neural network and PID controllers for studying the model, and proposes a solution for optimal control problems using these controllers. The results of computer experiments on trajectory dynamics are presented.
ARTIFICIAL INTELLIGENCE TRENDS IN SYSTEMS, VOL 2
(2022)
Article
Medicine, Research & Experimental
Yuwen Zhu, Yan Guo, Yujia Xue, Anqi Zhou, Ying Chen, Yifei Chen, Xiulian Miao, Fangqiao Lv
Summary: BRG1 plays an important role in HSC-myofibroblast transition and targeting it could be a reasonable strategy for liver fibrosis intervention.
Article
Medicine, Research & Experimental
Liu Ye, Beibei Liu, Jingling Huang, Xiaolin Zhao, Yuan Wang, Yungen Xu, Shuping Wang
Summary: Doublecortin-like kinase 1 (DCLK1) is a significant prooncogenic factor that is strongly associated with the malignant progression and clinical prognosis of various cancers. DCLK1 plays important roles in stem cell marker regulation, tumor cell reprogramming, and immune evasion. However, the exact biological functions of DCLK1, especially the disparities between its alpha- and beta-form transcripts in cancer progression, remain ambiguous.
Article
Medicine, Research & Experimental
Jiahui Yang, Xiaoyu Chen, Tianjing Liu, Yongyan Shi
Summary: This article reviews the role of bile acids in necrotizing enterocolitis (NEC) and their potential therapeutic value. The dysregulation of bile acids is associated with intestinal injury, and inflammatory factors in the liver also play a crucial role in regulating bile acid transport. The bile acid metabolic pathway is important for regulating intestinal microbiota, cell proliferation, and barrier protection.
Review
Medicine, Research & Experimental
Zhenzheng Zhu, Yuemiao Xu, Yuwei Xia, Xinru Jia, Yixin Chen, Yuyue Liu, Leyin Zhang, Hui Chai, Leitao Sun
Summary: Bile acid, as the final product of cholesterol breakdown, plays a complex regulatory and signaling role in human metabolism. Research suggests that it has the potential to enhance metabolism and regulate chronic metabolic diseases through various pathways. The interaction between bile acid and gut microbiota is also of great significance.
Article
Medicine, Research & Experimental
Xin He, Hong-Xu Zhou, Xian Fu, Kai-Di Ni, Ai-Zhi Lin, Ling-Tong Zhang, Hou-Hua Yin, Qing Jiang, Xue Zhou, Yi-Wen Meng, Jun-Yan Liu
Summary: DON exposure causes an increase in deoxycholic acid (DCA), which contributes to intestinal injury. DCA may be a potential therapeutic target for DON enterotoxicity.
Article
Medicine, Research & Experimental
Zhitao Wang, Heng Ma, Abdul Nasir, Sufang Liu, Zhisong Li, Feng Tao, Qian Bai
Summary: This study reveals the involvement of TET1-mediated epigenetic regulation in chronic TMJ pain through trigeminal TNF alpha signaling.
Article
Medicine, Research & Experimental
Lu Yu, Hao Ran, Yaru Lu, Qian Ma, Huan Huang, Weibin Liu
Summary: This study found that the HIF-1 alpha inhibitor BAY 87-2243 can alleviate the symptoms of the Experimental Autoimmune Myasthenia Gravis (EAMG) inflammation model. BAY 87-2243 can restore the balance of CD4(+)T cell subsets, reduce the production of pro-inflammatory cytokines, and act as both an immune imbalance regulator and anti-inflammatory.
Article
Medicine, Research & Experimental
Alex Peralvarez-Marin, Montse Sole, Judith Serrano, Alice Taddeucci, Belen Perez, Clara Penas, Gemma Manich, Marcel Jimenez, Pilar D'Ocon, Francesc Jimenez-Altayo
Summary: This study provides the first evidence that TRPV2 channels may modulate vascular tone by balancing opposing inputs from the endothelium and smooth muscle, leading to net vasodilation. The amplification of TRPV2 channel-induced activity by NO emphasizes the pathophysiological relevance of these findings.
Article
Medicine, Research & Experimental
Amin Ullah, Jing Zhao, Jiakun Li, Rajeev K. Singla, Bairong Shen
Summary: Gastric cancer is the fifth-most prevalent and second-most deadly cancer worldwide. Late onset of symptoms makes early detection important. CXC chemokines play an important role in the pathological process of gastric cancer, but their exact role in diagnosis and prognosis is not fully understood. Inhibiting CXC chemokines shows promise as a targeted therapy.
Article
Medicine, Research & Experimental
Menna S. Zeyada, Salma M. Eraky, Mamdouh M. El-Shishtawy
Summary: The current study demonstrates the prophylactic and antifibrotic effects of Trig against BLM-induced PF by targeting multiple signaling pathways. The combination of Trig and Pirf may be a promising approach to enhance Pirf's anti-fibrotic effect.