Spinocerebellar Ataxia [Working Title]最新文献

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Human Pluripotent Stem Cell-Derived Cerebellar Neurons: From Development to Modeling Cerebellar Ataxias 人类多能干细胞衍生的小脑神经元:从发育到模拟小脑共济失调

Spinocerebellar Ataxia [Working Title] Pub Date : 2021-04-28 DOI: 10.5772/INTECHOPEN.96653

Roxana Deleanu

{"title":"Human Pluripotent Stem Cell-Derived Cerebellar Neurons: From Development to Modeling Cerebellar Ataxias","authors":"Roxana Deleanu","doi":"10.5772/INTECHOPEN.96653","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.96653","url":null,"abstract":"The most affected cell types in cerebellar ataxias are the cerebellar neurons, which are not readily accessible for cellular and molecular investigation. Pluripotent stem cell (PSC) technology has emerged as an important tool for generating diverse types of neurons, which are used in order to better understand the human nervous system development and pathologies. In this chapter, the strategies for the differentiation of human PSCs toward cerebellar neurons are overviewed, followed by an outlook of their further optimization and diversification by implementing the knowledge from cerebellar development and new cell culture approaches. The optimization stategies are based on the recent progress made in defining the cell populations in mature and developing mouse and human cerebellum. The cellular phenotypes and organization in mouse and human cerebellum are briefly presented, followed by an overview of our current knowledge about their development, which includes pattering, proliferation, neurogenesis, gliogenesis, migration, connectivity and maturation. To date, however, relatively few studies have used induced PSCs (iPSCs) to model cerebellar ataxias and even fewer have looked directly to cerebellar neurons. The reported iPSC-derived in vitro models for cerebellar ataxias are reviewed, followed by an outlook of how to improve these models by generating and exporing the cerebellar neurons.","PeriodicalId":333408,"journal":{"name":"Spinocerebellar Ataxia [Working Title]","volume":"19 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124885787","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

Impact of Nutrition in Spinocerebellar Ataxia 营养对脊髓小脑共济失调的影响

Spinocerebellar Ataxia [Working Title] Pub Date : 2021-04-12 DOI: 10.5772/INTECHOPEN.96904

Donnette Wright, Kadiann Hewitt-Thompson

{"title":"Impact of Nutrition in Spinocerebellar Ataxia","authors":"Donnette Wright, Kadiann Hewitt-Thompson","doi":"10.5772/INTECHOPEN.96904","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.96904","url":null,"abstract":"This chapter explores the link between the health outcomes of spinocerebellar ataxia and diet and nutrition as well as overall quality of life and well-being that is achieved as a result of nutritional support and nutritional profile. Spinocerebellar ataxia is a hereditary condition characterized by degenerative changes to parts of the brain, extending to the spinal cord, that affects mobility and voluntary actions. Due to the deteriorating impact of this neurological disorder, the management of health and wellness of the individual is imperative in stemming physiological decline and morbidity. The connections between dietary intake, quality of life and well-being are important components of the health response in providing optimum health outcomes for clients diagnosed with spinocerebellar ataxia. Consequently, an examination of factors that impede, promote and generally affect dietary intake, nutritional status and profile is essential towards improving disease related quality of life and morbidity and mortality risk. The cyclical impact of the neurological condition on nutritional status and its corresponding impact on disease progression is an important exploratory point. Finally, recommendations and standardized guidance are crucial to expanding the health care approach and the overall wellness of individuals with spinocerebellar ataxia.","PeriodicalId":333408,"journal":{"name":"Spinocerebellar Ataxia [Working Title]","volume":"55 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114115356","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