Review
Biochemistry & Molecular Biology
Pavlina Hemerkova, Martin Valis
Summary: ALS is a neurodegenerative disease that affects motor neurons and currently has no cure. Free oxygen radicals are known to play a role in the pathogenesis of ALS, while antioxidant enzymes like SOD1 are crucial for antioxidant protection.
Article
Multidisciplinary Sciences
Li-Qiang Wang, Yeyang Ma, Han-Ye Yuan, Kun Zhao, Mu-Ya Zhang, Qiang Wang, Xi Huang, Wen-Chang Xu, Bin Dai, Jie Chen, Dan Li, Delin Zhang, Zhengzhi Wang, Liangyu Zou, Ping Yin, Cong Liu, Yi Liang
Summary: The authors determined the cryo-EM structure of SOD1 fibrils, providing insights into the conversion of SOD1 during the pathogenesis of ALS.
NATURE COMMUNICATIONS
(2022)
Article
Clinical Neurology
Chaofan Geng, Zhenzhen Yang, Pengfei Xu, Hongju Zhang
Summary: The study found that decreased serum vitamin B12 levels are independently associated with the development of narcolepsy, especially in male patients. Multivariate logistic regression analysis showed that serum vitamin B12 is independently associated with narcolepsy.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2022)
Article
Biochemistry & Molecular Biology
Venkatesan Santhanam, Priya Modi, Umesh K. Mishra, Ishrat Jahan, Namakkal G. Ramesh, Shashank Deep
Summary: In this study, the first iminosugar that inhibits superoxide dismutase fibrillation associated with ALS is reported. Novel triazole and tetrazole embedded iminosugars were successfully synthesized, and one of these designed iminosugars was found to inhibit SOD1 fibrillation and break pre-formed fibrils. Docking and MD simulation studies indicated that this compound interacts with the key residue Arg69 of SOD1 through hydrogen bonding.
INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES
(2023)
Article
Medicine, General & Internal
Teresa Cunha-Oliveira, Daniela Franco Silva, Luis Segura, Ines Baldeiras, Ricardo Marques, Tatiana Rosenstock, Paulo J. Oliveira, Filomena S. G. Silva
Summary: Distinct redox signatures were found in lymphoblasts from mutSOD1, undSOD1, and healthy controls, which can serve as therapeutic targets for ALS drug development. High heterogeneity in redox profiles between cohorts was observed, but clustering analysis successfully segregated healthy controls from ALS samples based on specific parameters. These findings provide valuable insights for understanding oxidative stress profiles in different forms of ALS and potential treatment strategies.
EUROPEAN JOURNAL OF CLINICAL INVESTIGATION
(2022)
Article
Clinical Neurology
Benjamin G. Trist, Sian Genoud, Stephane Roudeau, Alexander Rookyard, Amr Abdeen, Veronica Cottam, Dominic J. Hare, Melanie White, Jens Altvater, Jennifer A. Fifita, Alison Hogan, Natalie Grima, Ian P. Blair, Kai Kysenius, Peter J. Crouch, Asuncion Carmona, Yann Rufin, Stephane Claverol, Stijn Van Malderen, Gerald Falkenberg, David J. Paterson, Bradley Smith, Claire Troakes, Caroline Vance, Christopher E. Shaw, Safa Al-Sarraj, Stuart Cordwell, Glenda Halliday, Richard Ortega, Kay L. Double
Summary: This study examined the changes in SOD1 protein in post-mortem spinal cord tissues of ALS patients. The results showed mislocalization and accumulation of SOD1 protein in motor neurons of ALS patients, which was associated with instability and mismetallation of enzymatically active SOD1 dimers, as well as alterations to SOD1 post-translational modifications and molecular chaperones governing SOD1 maturation. These changes mostly occurred in regions of neurodegeneration and differentiated ALS patients from controls effectively.
Article
Biochemistry & Molecular Biology
Payam Baziyar, Bagher Seyedalipour, Saman Hosseinkhani
Summary: Neurotoxic species of misfolded hSOD1 are involved in the process of causing amyotrophic lateral sclerosis (ALS). The mechanism of this toxicity, resulted from gain-of-function, is still unknown. In this study, two point-mutations on metal-binding sites were investigated to understand their effects on ALS progress. The results showed increased tendency of amyloid aggregation, structural alterations, and increased hydrophobic surface pockets in the mutants. The findings support the hypothesis that mutation in the zinc-binding loop may trigger misfolding and fibrillar aggregation, leading to pathological changes in familial forms of ALS.
Article
Neurosciences
Teresa Cunha-Oliveira, Marcelo Carvalho, Vilma Sardao, Elisabete Ferreiro, Debora Mena, Francisco B. Pereira, Fernanda Borges, Paulo J. Oliveira, Filomena S. G. Silva
Summary: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with mitochondrial alterations in lymphoblasts that may have diagnostic and therapeutic implications.
MOLECULAR NEUROBIOLOGY
(2022)
Article
Clinical Neurology
Karin M. Forsberg, Karin S. Graffmo, Erica Stenvall, Naima Tabikh, Stefan L. Marklund, Thomas Brannstrom, Peter M. Andersen
Summary: The D90A mutation in the SOD1 gene is associated with atypical features in amyotrophic lateral sclerosis, including slowly evolving motor symptoms, recessive heredity, and potential involvement of sensory, autonomic, and urinary bladder functions. This study reveals that neuropathological changes in patients with homozygous D90A mutation extend beyond the motor system to include cognitive and sensory cortical areas, but do not affect non-nervous organs.
ACTA NEUROPATHOLOGICA
(2023)
Article
Clinical Neurology
Delia Gagliardi, Paolo Ripellino, Megi Meneri, Roberto Del Bo, Sara Antognozzi, Giacomo Pietro Comi, Claudio Gobbi, Antonia Ratti, Nicola Ticozzi, Vincenzo Silani, Dario Ronchi, Stefania Corti
Summary: In this study, the authors provided a clinical and molecular description of a cohort of SOD1-ALS patients, revealing the heterogeneity in clinical and molecular characteristics of SOD1 mutations. The cohort exhibited variable expressivity, atypical presentations, and different modes of inheritance. With the availability of SOD1-directed antisense oligonucleotide for SOD1-ALS patients, prompt screening for SOD1 mutations in ALS patients is recommended.
FRONTIERS IN NEUROLOGY
(2023)
Review
Psychiatry
Sirui Yan, Hailong Liu, Yaqiong Yu, Nashu Han, Wenzhi Du
Summary: This meta-analysis shows that high levels of homocysteine, low levels of vitamin B12, and normal folate level are closely correlated with OCD.
FRONTIERS IN PSYCHIATRY
(2022)
Article
Andrology
Jiangnan Xu, ZeKun Xu, Nianxin Ge, Chao Wang, Can Hu, Ze Chen, Jun Ouyang, Changsong Pei
Summary: The study found that ED patients had lower serum levels of folic acid and vitamin B12, and higher levels of homocysteine, with more pronounced differences in younger patients. Serum folic acid decreased with the severity of ED, while homocysteine increased. Multivariate analysis showed that serum folic acid and homocysteine significantly related to ED.
Review
Cell Biology
Ilaria Martinelli, Elisabetta Zucchi, Cecilia Simonini, Giulia Gianferrari, Giovanna Zamboni, Marcello Pinti, Jessica Mandrioli
Summary: Although mutations in the SOD1 gene account for only a minority of ALS cases, the discovery of this gene has greatly expanded our understanding of the diverse pathogenic basis of ALS. This review focuses on cognitive impairment in SOD1-ALS patients and highlights the potential frailty of frontal lobe function in patients with different SOD1-ALS mutations. Thoroughly reviewing the reported mutations could contribute to a comprehensive genotype-phenotype correlation database for SOD1-ALS.
NEURAL REGENERATION RESEARCH
(2023)
Article
Medicine, General & Internal
Erum Afaq, Anwar Ali, Rabia Jamil, Hira Fatima Waseem
Summary: This study investigated the relationship between serum homocysteine, folic acid, and vitamin-B12 levels and recurrent pregnancy loss (RPL). The results showed that reduced levels of serum folic acid and vitamin B12 were significantly associated with RPL cases, while homocysteine levels were slightly elevated but not significantly different. Supplementing folic acid and vitamin B12 before the next pregnancy in RPL patients is likely to improve pregnancy outcomes.
PAKISTAN JOURNAL OF MEDICAL SCIENCES
(2023)
Article
Pharmacology & Pharmacy
Tomoyuki Ueda, Taisei Ito, Masatoshi Inden, Hisaka Kurita, Akihito Yamamoto, Isao Hozumi
Summary: This study investigated the potential of stem cells from human exfoliated deciduous teeth (SHED) for the treatment of ALS. The study found that SHED-conditioned medium (CM) significantly reduced mutant SOD1-induced intracellular aggregates and neurotoxicity. The neuroprotective effects of SHED-CM were partly attributed to heat shock protein and the activation of insulin-like growth factor-1 receptor. In addition, SHED-CM also showed a protective effect on induced pluripotent stem cell-derived motor neurons and was effective against both familial and sporadic ALS. These findings suggest that SHED-CM could be a promising treatment for slowing the progression of ALS.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Clinical Neurology
You Ni, Dingding Shen, Ying Zhang, Yaying Song, Yining Gao, Qinming Zhou, Lu He, Dou Yin, Ying Wang, Fan Song, Meiyuan Chen, Yajun Lian, Yuan Chen, Xing Zhao, Xiang Zhang, Xiangjun Chen, Yuting Wang, Ling Zhang, Nanxun Mo, Dong Lv, Jun Liu, Zhifeng Mao, Lisheng Peng, Sheng Chen
Summary: The clinical spectrum of anti-IgLON5 disease is diverse, with symptoms including sleep disturbances, cognitive impairment, and movement disorders. Some patients may have parainfectious causes, and coexisting neural autoantibodies or other autoimmune diseases were identified in some cases. Most patients showed improvement with immunotherapy, and overall clinical outcomes were favorable for the majority of patients.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
You Ni, Zhao Yang, Qinming Zhou, Jun Liu, Haiyan Zhou, Sheng Chen
Summary: Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder with diverse clinical manifestations and difficulties in diagnosis. Chinese NIID patients have clinical features including an average onset age of 60.18 years, average duration of 4.06 years, and tremor as the most common initial symptom. MRI findings show high-intensity U-fibers signals, focal leukoencephalopathy, and cortical swelling. Reversible asymmetric leukoencephalopathy can also indicate the disease.
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
(2023)
Article
Neurosciences
Xiaojiao Xu, Jingjing Zhang, Song Li, Murad Al-Nusaif, Qinming Zhou, Sheng Chen, Weidong Le
Summary: The study found that neuroinflammation in the early pathogenesis of ALS is associated with increased immune-related genes, and BST2 may serve as a potential target for ameliorating microglia-mediated neuroinflammation pathologies in ALS.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Oncology
Qinming Zhou, Lu He, Jin Hu, Yining Gao, Dingding Shen, You Ni, Yuening Qin, Huafeng Liang, Jun Liu, Weidong Le, Sheng Chen
Summary: The role of CORO1A in ALS pathogenesis was discovered, potentially affecting the disease onset and progression by blocking autophagic flux. Therefore, CORO1A might be a potential biomarker and therapeutic target for ALS.
FRONTIERS OF MEDICINE
(2022)
Article
Immunology
You Ni, Yifan Feng, Dingding Shen, Ming Chen, Xiaona Zhu, Qinming Zhou, Yining Gao, Jun Liu, Qi Zhang, Yuntian Shen, Lisheng Peng, Zike Zeng, Dou Yin, Ji Hu, Sheng Chen
Summary: The study demonstrated that anti-IgLON5 antibodies can induce progressive and irreversible behavioral deficits in mice, supporting their pathogenicity in vivo. The effects of these antibodies include cognitive abnormalities, increased brain-bound antibodies, increased microglial and astrocyte density, and altered synaptic homeostasis.
JOURNAL OF NEUROINFLAMMATION
(2022)
Editorial Material
Neurosciences
Wenting Guo, Zhang-Yu Zou, Yue Leng, Ding-Ding Shen, Sheng Chen
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2022)
Article
Clinical Neurology
Yizongheng Zhang, Qinming Zhou, Lisheng Peng, Sheng Chen
ANNALS OF NEUROLOGY
(2023)
Review
Cell Biology
Yi-Zong Heng Zhang, You Ni, Yi-Ning Gao, Ding-Ding Shen, Lu He, Dou Yin, Huan-Yu Meng, Qin-Ming Zhou, Ji Hu, Sheng Chen
Summary: Anti-IgLON5 disease is a complex autoimmune disorder of the nervous system associated with autoantibodies against IgLON5. It has characteristics of both autoimmunity and neurodegeneration, and the neuronal damage induced by IgLON5 antibody is irreversible. The disease is difficult to diagnose and treat, and further research is needed to better understand it.
NEURAL REGENERATION RESEARCH
(2023)
Article
Immunology
Qinming Zhou, Dou Yin, Jianfang Ma, Sheng Chen
Summary: This study presents three cases of autoimmune encephalitis (AE) treated with ofatumumab (OFA), an anti-CD20 antibody. OFA was administered subcutaneously at a dose of 20 mg two or three times within three weeks. The patients had mild adverse effects but showed favorable responses with reduced antibody titer and clinical symptom improvement. During a three-month follow-up, their symptoms remained stable or even improved. OFA injection is demonstrated to be a safe and effective therapeutic option for treating AE.
JOURNAL OF NEUROIMMUNOLOGY
(2023)
Letter
Clinical Neurology
Dingding Shen, Qinming Zhou, Huanyu Meng, Min Zhang, Lisheng Peng, Sheng Chen
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Sijia Du, Cheng Yuan, Qinming Zhou, Xinyun Huang, Hongping Meng, Meidi Chen, Hanzhong Wang, Qiu Huang, Suncheng Xiang, Dahong Qian, Biao Li, Sheng Chen, Min Zhang
Summary: In this study, a deep learning-based method was developed to extract radiomics features from PET and MR images for prediction of ARR in MS patients. The results showed that the deep learning and PET/MR radiomics-based model is an effective tool in assisting ARR classification of MS patients.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Article
Biochemistry & Molecular Biology
Yining Gao, Hongxia Li, Huoqing Luo, You Ni, Yifan Feng, Lu He, Qinming Zhou, Ji Hu, Sheng Chen
Summary: This study investigated the underlying mechanisms of movement disorders caused by anti-IgLON5 antibodies. The results showed that mice injected with these antibodies exhibited persistent movement impairments for up to 3 months, which may be related to impaired dopaminergic pathway and neurodegenerative changes induced by the antibodies.
Article
Health Care Sciences & Services
Zhiguo Li, Chao Zhang, Ting Chang, Xinghu Zhang, Huan Yang, Feng Gao, Jinzhou Feng, Hongbo Liu, Sheng Chen, Lihua Wang, Chunsheng Yang, Huining Li, Yuesong Pan, Jacqueline Palace, Fu-Dong Shi
Summary: This prospective multicenter cohort study evaluated the diagnostic accuracy of cell-based assay (CBA), radioimmunoprecipitation assay (RIPA), and enzyme-linked immunosorbent assay (ELISA) in detecting AChR and MuSK autoantibodies for the diagnosis of myasthenia gravis (MG).
LANCET REGIONAL HEALTH-WESTERN PACIFIC
(2023)
Article
Immunology
Ruinan Shen, Dingding Shen, Qinming Zhou, Min Zhang, Sheng Chen
Summary: The SARS-CoV-2 vaccine is considered the most effective method to prevent infection, but in very rare cases, autoimmune neurological diseases may occur. This article reports three rare cases of autoimmune encephalitis with definite auto-antibody after SARS-CoV-2 vaccination, all of whom had good prognosis after treatment. 18F-DPA-714 PET/MRI may be a powerful tool for quantitative analysis of neuroinflammation in patients of autoimmune encephalitis.
BRAIN, BEHAVIOR, & IMMUNITY - HEALTH
(2022)
Article
Neurosciences
Huanyu Meng, Shuyu Zheng, Shaicun Yuan, Qinming Zhou, Yining Gao, You Ni, Lu He, Dou Yin, Min Zhang, Sheng Chen
Summary: This study reports a method using F-18-florbetapir positron emission tomography-magnetic resonance imaging (PET/MRI) to evaluate myelin recovery in GFAP-A patients. The study found that after treatment, there was a significant increase in F-18-florbetapir uptake in the brain lesions of the patient, along with a reduced disability score.
TRANSLATIONAL NEUROSCIENCE
(2022)
Correction
Neurosciences
Lucia Privitera, Ellen L. Hogg, Matthias Gaestel, Mark J. Wall, Sonia A. L. Correa
Article
Neurosciences
Li-Ya Jiang, Guan-Hao Wang, Jing-Jiao Xu, Xiao-Li Li, Xiao-Yan Lin, Xiang Fang, Hong-Xu Zhang, Mei Feng, Chun-Ming Jiang
Summary: This study reveals the importance of LINC00473 in regulating temozolomide (TMZ) resistance in glioblastoma (GB) and its potential mechanism. By regulating the expression of CEBP alpha and MGMT, LINC00473 promotes the formation of chemoresistance. Furthermore, LINC00473 can transfer chemoresistance to adjacent sensitive cells through exosomes.
Article
Neurosciences
Olga Kopach, Tetyana Pivneva, Nataliya Fedirko, Nana Voitenko
Summary: This study found that diabetic animals exhibit severe xerostomia characterized by reduced saliva flow rate, diminished total protein content, and decreased amylase activity. The impaired saliva production in diabetes is associated with reduced and delayed intracellular Ca2+ signals in submandibular acinar cells, caused by malfunctioning mitochondria. Targeting malfunctioning mitochondria may be a potential strategy for the treatment of diabetic xerostomia.
Article
Neurosciences
Nicholas M. Timme, Cherish E. Ardinger, Seth D. C. Weir, Rachel Zelaya-Escobar, Rachel Kruger, Christopher C. Lapish
Summary: This study aimed to assess aversion-resistant drinking behavior in head-fixed mice and explore the relationship between non-consummatory behaviors and aversion-resistant drinking. The results showed that head-fixed mice exhibited heterogenous levels of aversion-resistant drinking and non-consummatory behaviors were related to the intensity of this behavior.
Article
Neurosciences
David R. Maguire, Charles P. France
Summary: Methocinnamox (MCAM) is a novel, long-acting opioid receptor antagonist that effectively decreases fentanyl self-administration and prevents opioid overdose in monkeys. The study demonstrates the potential therapeutic utility of MCAM in the treatment of opioid use disorder.
Article
Neurosciences
Xiang Li, Dan Feng, Shenglu Ma, Mingxing Li, Shulei Zhao, Man Tang
Summary: This study investigated the effects of fluoxetine on neurochemical, neurobiological, and neurobehavioral changes in different subregions of the hippocampus. The results showed that fluoxetine increased dialysate 5-HT, decreased membrane 5-HTT protein, and increased cytoplasmic fraction. Additionally, fluoxetine reduced immobility times in behavioral tests, with greater effects observed in the ventral subregion compared to the dorsal subregion.
Article
Neurosciences
Alexander V. Zholos, Mariia I. Melnyk, Dariia O. Dryn
Summary: Acetylcholine is an important neurotransmitter in visceral smooth muscles, activating M2 and M3 muscarinic receptors to cause smooth muscle excitation and contraction. This review focuses on the cellular and molecular mechanisms underlying acetylcholine-induced depolarisation and smooth muscle contraction, as well as the effects of anticholinergic drugs on gastrointestinal motility. The knowledge gained from recent studies has greatly expanded our understanding of these processes.
Article
Neurosciences
Zhenlong Li, Hsien-Yu Peng, Chau-Shoun Lee, Tzer-Bin Lin, Ming-Chun Hsieh, Cheng-Yuan Lai, Han-Fang Wu, Lih-Chyang Chen, Mei-Ci Chen, Dylan Chou
Summary: Methylone shows significant efficacy in treating depression and social deficits, making it an ideal candidate for anti-depressant medication.
Article
Neurosciences
Aline Freyssin, Allison Carles, Sarra Guehairia, Gilles Rubinstenn, Tangui Maurice
Summary: This study explores the potential of combining FENM and S1R agonists in the treatment of Alzheimer's disease. The results showed that most FENM-based combinations can protect against learning deficits caused by A beta 25-35, with better efficacy in short-term memory.
Article
Neurosciences
J. D. Lorente, J. Cuitavi, L. Rullo, S. Candeletti, P. Romualdi, L. Hipolito
Summary: This study analyzed the effects of pain on negative affect in different sexes and time courses, as well as the involvement of the dynorphinergic and corticotropin releasing factor systems in these pain-related behaviors. The results showed sex and time-dependent anxiety- and anhedonia-like behaviors induced by pain in female rats. The recruitment of KOR/DYN in the NAc was identified as a key neurological substrate mediating pain-induced behavioral alterations.
Article
Neurosciences
Rongjun Liu, Daofan Sun, Xiuzhong Xing, Qingge Chen, Bo Lu, Bo Meng, Hui Yuan, Lan Mo, Liufang Sheng, Jinwei Zheng, Qiusheng Wang, Junping Chen, Xiaowei Chen
Summary: The coexistence of pain and depression is frequently observed in patients with chronic pain and depression. Oxytocin, a neuropeptide, has been reported to relieve chronic pain and depressive symptoms. This study investigated the effect of intranasal oxytocin on neuropathic pain and comorbid depressive symptoms, and found that oxytocin attenuated depression-like behavior but did not alleviate mechanical hyperalgesia. The results suggest that intranasal oxytocin may have the potential to treat depressive symptoms in neuropathic pain patients.
