Novel Aspects on Motor Neuron Disease最新文献

Introductory Chapter: Introduction to Novel Aspects on Motor Neuron Disease 导论章:运动神经元疾病新方面的介绍

Novel Aspects on Motor Neuron Disease Pub Date : 2020-05-20 DOI: 10.5772/intechopen.92610

H. F. Sibat, L. Valdés

引用次数: 0

Introduction to Novel Motor Neuron Disease 新型运动神经元疾病导论

Novel Aspects on Motor Neuron Disease Pub Date : 2020-04-25 DOI: 10.5772/intechopen.91921

H. F. Sibat, L. Valdés

{"title":"Introduction to Novel Motor Neuron Disease","authors":"H. F. Sibat, L. Valdés","doi":"10.5772/intechopen.91921","DOIUrl":"https://doi.org/10.5772/intechopen.91921","url":null,"abstract":"Motor neuron disease (MND) is a progressive and fatal neuromuscular disease; the most common and severe form of MND presentation is amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease. The majority of ALS patients die within 2–5 years of receiving a diagnosis. Familial ALS is a hereditary form of the disease and accounts for 5–10% of cases, whereas the remaining cases have no clearly defined etiology. ALS affects persons of all ethnicities and races; currently, no curative treatment for ALS is available worldwide. ALS is also the major adult-onset MND and is clinically, pathologically, and genetically associated with fronto-temporal dementia in some cases, which is the second cause of dementia in elderly people. However, MND does not affect sphincter, sexual function, or eye movements. MND is the most common degenerative disorder affecting the upper and lower motor neurons at the same time. Most of the patients presenting MND in our series complained of muscle weakness, muscle wasting, fasciculation, and spasticity plus lower cranial nerve disturbances. According to our bibliographic studies, apart from nusinersen, it seems to be that riluzole and edaravone also improve motor neuron function by acting on SK channels.","PeriodicalId":396133,"journal":{"name":"Novel Aspects on Motor Neuron Disease","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126873713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Stem Cell Therapy in Motor Neuron Disease 运动神经元疾病的干细胞治疗

Novel Aspects on Motor Neuron Disease Pub Date : 2019-10-23 DOI: 10.5772/intechopen.87116

A. Sharma, H. Sane, N. Gokulchandran, P. Badhe, A. Paranjape, Radhika Pradhan, Rohit Das, H. Biju

{"title":"Stem Cell Therapy in Motor Neuron Disease","authors":"A. Sharma, H. Sane, N. Gokulchandran, P. Badhe, A. Paranjape, Radhika Pradhan, Rohit Das, H. Biju","doi":"10.5772/intechopen.87116","DOIUrl":"https://doi.org/10.5772/intechopen.87116","url":null,"abstract":"Motor neuron disease (MND) is an insidious, fatal disorder that progresses with the selective loss of anterior horn cells of the spinal column. Over 150 years since it was first described, various therapeutic approaches have been tested in the quest of a cure but with little success. Current standard therapy only improves lifespan by a few months; palliative care is the only option available for patients. Stem cell therapy is a potent approach for the treatment of this devastating disease. A multi-tude of vitalizing effects, both paracrine and somatic, a robust safety profile, as well as ease of availability make a strong case for using these cells for therapeutic purposes. Coupled with rigorous rehabilitation, this powerful treatment modality has been shown to slow disease progression, improve quality of life, and increase survival, along with being well tolerated by amyotrophic lateral sclerosis (ALS)/ MND patients. Compelling preclinical as well as clinical evidence abounds that stem cells hold great potential as a therapy for ALS/MND. Although not a definitive solution yet, stem cells have been verified to have slowed and/or halted disease progression in a subset of ALS/MND patients.","PeriodicalId":396133,"journal":{"name":"Novel Aspects on Motor Neuron Disease","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133675937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Novel Aspects on Motor Neuron Disease: The Recent Genetic Studies on ALS 运动神经元疾病的新进展:ALS基因研究进展

Novel Aspects on Motor Neuron Disease Pub Date : 2019-07-09 DOI: 10.5772/intechopen.82085

Junling Wang

引用次数: 1

Contribution of Aberrant Astrocytes to Motor Neuron Damage and Death in the SOD1G93A Rat Experimental Model of ALS 异常星形胶质细胞在SOD1G93A肌萎缩侧索硬化症大鼠实验模型运动神经元损伤和死亡中的作用

Novel Aspects on Motor Neuron Disease Pub Date : 2019-04-03 DOI: 10.5772/INTECHOPEN.84695

Gabriel Otero Damianovich, Olga Cristina Parada, Pablo Díaz-Amarilla, Eugenia Isasi, Carmen Isabel Bolatto Pereira, S. Olivera-Bravo

{"title":"Contribution of Aberrant Astrocytes to Motor Neuron Damage and Death in the SOD1G93A Rat Experimental Model of ALS","authors":"Gabriel Otero Damianovich, Olga Cristina Parada, Pablo Díaz-Amarilla, Eugenia Isasi, Carmen Isabel Bolatto Pereira, S. Olivera-Bravo","doi":"10.5772/INTECHOPEN.84695","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.84695","url":null,"abstract":"Amyotrophic lateral sclerosis (ALS) is an incurable paralyzing disease characterized by motor neuron death and glial reactivity. Superoxide dismutase 1 (SOD1) are among the most frequent alterations found in around 15–20% of ALS inheritable forms. Mutant SOD1 murine models mimic main human ALS features and allow purposing that pathological mechanisms include defective communication between neural cells together with astrocyte preponderant roles in disease progression. Years ago, a subset of the most neurotoxic aberrant astrocytes (AbAs) was obtained from spinal cords of SOD1G93A rats. AbA cultures show an exponential growing yield since the early symptoms of the disease up to the terminal stages. In cultures, AbAs present unprecedented toxicity to motor neurons, increased proliferation, loss of mature astrocyte markers, as well as extreme ER stress and abundant extracellular matrix components. Strikingly, AbA phenotype seems to be changing along few passages suggesting its signaling and features may accompany disease progression. However, the link between main AbA features and their highest motor neuron toxicity is not yet completely understood. Here, we reviewed ALS underlying pathological mechanisms in association to AbA phenotype, to collaborate with identification of the most relevant processes that seem crucially involved in the triggering or maintenance of neurotoxicity.","PeriodicalId":396133,"journal":{"name":"Novel Aspects on Motor Neuron Disease","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127148031","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Structural and Functional Consequences of the SMA-Linked Missense Mutations of the Survival Motor Neuron Protein: A Brief Update 存活运动神经元蛋白的sma相关错义突变的结构和功能后果:简要更新

Novel Aspects on Motor Neuron Disease Pub Date : 2018-11-05 DOI: 10.5772/INTECHOPEN.81887

Wei Li

引用次数: 1