Review
Medicine, General & Internal
Sarah L. Boddy, Ilaria Giovannelli, Matilde Sassani, Johnathan Cooper-Knock, Michael P. Snyder, Eran Segal, Eran Elinav, Lynne A. Barker, Pamela J. Shaw, Christopher J. McDermott
Summary: The gut microbiome may play a significant role in the pathogenesis of ALS, with research indicating that microbial changes can affect disease outcomes. Variability in gut microbiota between individuals may explain the different disease outcomes seen in ALS patients.
Review
Immunology
Sarah Martin, Carolina Battistini, Jun Sun
Summary: Amyotrophic lateral sclerosis (ALS) is a debilitating disease characterized by motor neuron degeneration, and its etiology and pathophysiology remain poorly understood. Recent studies suggest the significant roles of intestinal dysfunction and microbiota in ALS development and progression. However, research on innate immunity and intestinal changes in early disease pathology is lacking.
FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY
(2022)
Review
Cell Biology
T. J. Tracey, S. E. Kirk, F. J. Steyn, S. T. Ngo
Summary: Lipids are essential for cellular health and maintenance in the central nervous system (CNS), playing roles in structural integrity, signaling, and metabolism. Changes in lipid metabolism in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), can impact key cellular processes, warranting further research.
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
(2021)
Article
Clinical Neurology
Ji He, Jiayu Fu, Wei Zhao, Chuan Ren, Ping Liu, Lu Chen, Dan Li, Lu Tang, Lequn Zhou, Yixuan Zhang, Xinran Ma, Gaoqi Zhang, Nan Li, Dongsheng Fan
Summary: This study found a significant increase in the prevalence and degree of hypermetabolism in ALS patients compared to matched controls in China. The metabolic index was closely associated with disease progression and body composition, serving as an independent prognostic indicator for worse survival in ALS.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Kailin Xia, Simon Witzel, Christina Witzel, Veronika Klose, Dongsheng Fan, Albert C. Ludolph, Johannes Dorst
Summary: This study identified distinct metabolic profiles in presymptomatic ALS gene carriers, which might be associated with disease progression in the symptomatic phase.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Medicine, General & Internal
Saumya Shah, Alberto Locca, Yair Dorsett, Claudia Cantoni, Laura Ghezzi, Qingqi Lin, Suresh Bokoliya, Hunter Panier, Cassandra Suther, Matthew Gormley, Yue Liu, Emily Evans, Robert Mikesell, Kathleen Obert, Amber Salter, Anne H. Cross, Phillip I. Tarr, Amy Lovett-Racke, Laura Piccio, Yanjiao Zhou
Summary: This study revealed alterations in the gut mycobiome of patients with multiple sclerosis compared to healthy controls, with Saccharomyces and Aspergillus being over-represented in the patient group. Different mycobiome profiles were associated with specific bacterial microbiome and immune cell subsets in the blood of patients. Further research is needed to explore the potential causal relationship between the mycobiome and multiple sclerosis, as well as its interactions with bacteria and autoimmunity.
Article
Immunology
Wenxia Jiang, Lei Gong, Fang Liu, Yikun Ren, Jun Mu
Summary: The study found that there were significant changes in the microbial composition and metabolism of the PSD rats, particularly related to lipid metabolism.
FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY
(2021)
Article
Neurosciences
Shuangwu Liu, Yuying Zhao, Qingguo Ren, Dong Zhang, Kai Shao, Pengfei Lin, Ying Yuan, Tingjun Dai, Yongqing Zhang, Ling Li, Wei Li, Peiyan Shan, Xiangshui Meng, Qian Wang, Chuanzhu Yan
Summary: This study investigated amygdala abnormalities in ALS patients, revealing distinct patterns at different clinical disease stages and highlighting their impact on anxiety and cognitive dysfunction.
HUMAN BRAIN MAPPING
(2022)
Article
Neurosciences
Pan Liu, Yongxiang Tang, Wanzhen Li, Zhen Liu, Ming Zhou, Jian Li, Yanchun Yuan, Liangjuan Fang, Jifeng Guo, Lu Shen, Hong Jiang, Beisha Tang, Shuo Hu, Junling Wang
Summary: This study investigated the brain metabolic signature in Chinese amyotrophic lateral sclerosis (ALS) patients, finding hypometabolic clusters in the midbrain and cerebellum as compared to healthy controls. Furthermore, ALS patients showed hypometabolism in the bilateral temporal lobe and precentral gyrus, as well as hypermetabolism in the left anterior cingulate and occipital lobe. Genetic ALS patients exhibited specific brain metabolic patterns and a higher incidence of sensory disturbance compared to non-genetic ALS patients. This study provides unprecedented evidence of ALS-related brain metabolic changes and highlights the role of genetic factors in affecting brain metabolism and sensory disturbance risk in ALS.
CNS NEUROSCIENCE & THERAPEUTICS
(2023)
Review
Cell Biology
Alexandra Bouscary, Cyril Quessada, Frederique Rene, Michael Spedding, Bradley J. Turner, Alexandre Henriques, Shyuan T. Ngo, Jean-Philippe Loeffler
Summary: Sphingolipids play crucial roles in the development and maintenance of the nervous system, with dysregulated metabolism being linked to specific neurodegenerative diseases. Current research primarily focuses on ALS, seeking potential treatments that target sphingolipid metabolism in neurodegenerative diseases.
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
(2021)
Article
Clinical Neurology
Serena Lattante, Mario Sabatelli, Giulia Bisogni, Giuseppe Marangi, Paolo Niccolo Doronzio, Francesco Martello, Anna Gloria Renzi, Elda Del Giudice, Alberta Leon, Paola Cimbolli, Daniela Marchione, Umberto Costantino, Gabriele Lucioli, Daniela Bernardo, Emiliana Meleo, Agata Katia Patanella, Angela Romano, Marcella Zollino, Amelia Conte
Summary: This study assessed the contribution of TARDBP gene variants in a large cohort of Italian ALS patients and identified 13 rare variants, with p.A382T and p.G294V being the most prevalent. Cognitive impairment was found in almost 30% of patients. There was marked clinical heterogeneity, especially for the p.A382T variant, suggesting a multifactorial/oligogenic pathogenic mechanism.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Biochemistry & Molecular Biology
Katarina Maksimovic, Mohieldin Youssef, Justin You, Hoon-Ki Sung, Jeehye Park
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects motor neurons, leading to muscle weakness, paralysis, and eventual death. Recent research has shown that ALS is not only limited to motor neurons, but also involves systemic metabolic dysfunction. This review examines the metabolic dysfunction in ALS at various levels, including muscle tissue, adipose tissue, liver, pancreas, and the central nervous system. It also discusses the future prospects of metabolic research in ALS and potential treatment options.
Article
Neurosciences
Bruno Costa Gomes, Nuno Peixinho, Rita Pisco, Marta Gromicho, Ana Catarina Pronto-Laborinho, Jose Rueff, Mamede de Carvalho, Antonio Sebastiao Rodrigues
Summary: Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease that causes muscle control loss, muscle atrophy, and death. This study aimed to assess the differential expression of plasma miRNAs in ALS patients and control populations. Although no specific miRNA signature for ALS diagnosis was identified, miR-7-2-3p, miR-26a-1-3p, miR-224-5p, and miR-206 were found to be potential candidates for understanding the pathophysiology of ALS.
MOLECULAR NEUROBIOLOGY
(2023)
Review
Medicine, General & Internal
Jiangwei Sun, Tingting Huang, Justine W. Debelius, Fang Fang
Summary: ALS is a rare and incurable neurodegenerative disease characterized by a loss of motor neurons in the brain and spinal cord. Contributions from outside the central nervous system to the etiology of ALS, including the gut microbiome, are increasingly recognized. Results from animal studies on altered gut microbiome composition in ALS are relatively consistent, while findings from human studies are largely inconclusive.
JOURNAL OF INTERNAL MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
George Anderson
Summary: The article integrates data on the biological underpinnings of ALS, focusing on the mitochondrial melatonergic pathway and systemic factors. Suppression of this pathway leads to changes in muscle brain-derived neurotrophic factor, contributing to a lack of metabolic trophic support at the neuromuscular junction. Activation of astrocytes and microglia is a driver of motor neuron susceptibility in ALS. Gut dysbiosis/permeability and pineal melatonin play important roles in upregulating the melatonergic pathway. The occupational risk of ALS in farmers, gardeners, and sportsmen/women is linked to exposure to glyphosate-based herbicides.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Zhe-Ren Tan, Xiao-Yan Long, Guo-Liang Li, Jin-Xia Zhou, Lan Long
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2016)
Article
Multidisciplinary Sciences
Ding Liu, Yumiao Zhang, Yu Wang, Chanjuan Chen, Xin Li, Jinxia Zhou, Zhi Song, Bo Xiao, Kevin Rasco, Feng Zhang, Shu Wen, Guoliang Li
SCIENTIFIC REPORTS
(2016)
Article
Clinical Neurology
Hui Li, Jinxia Zhou, Zongwei Yue, Li Feng, Zhaohui Luo, Si Chen, Xiaosu Yang, Bo Xiao
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2017)
Article
Clinical Neurology
Qihua Chen, Jinxia Zhou, Cao Huang, Bo Huang, Fangfang Bi, Hongxia Zhou, Bo Xiao
CURRENT NEUROVASCULAR RESEARCH
(2018)
Letter
Clinical Neurology
Xiaoliang Zhou, Wenbin Zhou, Jinxia Zhou, Lili Long, Bo Xiao
NEUROLOGICAL SCIENCES
(2015)
Article
Clinical Neurology
Si Chen, Qiao Liao, Ke Lu, Jinxia Zhou, Cao Huang, Fangfang Bi
CURRENT NEUROVASCULAR RESEARCH
(2020)
Article
Clinical Neurology
Yujiao Fu, Chaojun Zhou, Rui Song, Jinxin Peng, Xiaosu Yang, Bo Xiao, Jinxia Zhou, Hongyu Long
NEUROLOGICAL SCIENCES
(2020)
Article
Multidisciplinary Sciences
Qihua Chen, Na Deng, Ke Lu, Qiao Liao, Xiaoyan Long, Deming Gou, Fangfang Bi, Jinxia Zhou
Summary: Elevated levels of miR-133b and miR-221-3p showed high sensitivity and specificity in distinguishing early-stage PD patients from controls, suggesting their potential as biomarkers for early PD. Additionally, miR-4454 demonstrated the ability to differentiate between PD and MSA. These findings highlight the potential of miRNAs as non-invasive biomarkers for PD diagnosis.
SCIENTIFIC REPORTS
(2021)
Letter
Neurosciences
Min Zhang, Shujuan Meng, Mohammad al Mahmoud, Ye Li, Yuwei Dai, Chunhui Li, Jinxia Zhou, Bo Xiao, Lili Long
CNS NEUROSCIENCE & THERAPEUTICS
(2022)
Article
Clinical Neurology
Tianxin Yao, Qianqian Zeng, Yuanyuan Xie, Fangfang Bi, Le Zhang, Bo Xiao, Jinxia Zhou
Summary: This retrospective study examined the clinical and neuroimaging features of adult myelin oligodendrocyte glycoprotein (MOG) antibody-associated cortical encephalitis, as well as treatment response and outcomes. The results showed that MOG antibody-associated cortical encephalitis is common in adults, with clinical symptoms including fever, headache, and seizures. Neuroimaging findings were associated with bilateral involvement of the cortex and corpus callosum. The majority of patients had favorable outcomes.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2022)
Article
Neurosciences
Guanzhong Shi, Jinxia Zhou, Kun Huang, Fang-Fang Bi
Summary: This study provides an overview of the publishing characteristics and trends in ALS research since 2000 using a bibliometric analysis. The results show a steady increase in the output of ALS research, with the United States and Western Europe leading in this field.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Clinical Neurology
Qiao Liao, Si-Zhuo Li, Qian-Qian Zeng, Jin-Xia Zhou, Kun Huang, Fang-Fang Bi
Summary: Serum albumin concentration at admission and the END-IT score are independent predictive factors for short-term outcome in SE patients. A lower albumin concentration and higher END-IT score indicate an unfavorable outcome. Serum albumin concentration is comparable to the END-IT score in predicting functional outcome at discharge.
EPILEPTIC DISORDERS
(2023)
Article
Clinical Neurology
Jinxia Zhou, Qianqian Zeng, Qiao Liao, Qi Niu, Wenping Gu, Dandan Su, Sizhuo Li, Bo Xiao, Fangfang Bi
Summary: This study compared the protein expression in the cerebrospinal fluid (CSF) of bulbar-onset and spinal-onset ALS patients, and identified two potential biomarkers, LBP and HLADRA, associated with disease severity and survival. These findings may have implications for the diagnosis and treatment of ALS.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2023)
Review
Clinical Neurology
Kangzhi Chen, Yefan Lv, Xiaoyan Long, Weiping Liu, Jinxia Zhou
Summary: Sleep disorders and cognitive dysfunction are on the rise, with insomnia and Alzheimer's disease being the most common forms. Studies have shown a link between altered sleep patterns and cognitive impairment, with managing disrupted sleep patterns potentially beneficial in the absence of a cure for AD. This review focuses on exploring altered sleep patterns in insomnia as potential biomarkers for cognitive decline and investigating therapeutic targets based on research evidence.
CURRENT ALZHEIMER RESEARCH
(2021)