iPS Cells and Spinocerebellar Ataxia

Shum, Carole; Nishimura, Agnes L.

doi:10.1007/978-3-319-18314-5_4

Cited by 2 publications

(1 citation statement)

References 94 publications

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“…develop. Additional mechanistic understanding of the network of events produced by the mutation is crucial for the development of effective therapies, as none of the cerebellar ataxias is yet curable, treatable or preventable [143,145,[147][148][149].…”

Section: Ipsc-derived Models For Cerebellar Ataxiasmentioning

confidence: 99%

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

2022

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She has experience in innovative research for the development of new technologies and neurosciences. She is also an academic editor and reviewer of several scientific publications about neurological diseases. PrefaceSpinocerebellar ataxia (SCA) is a neurological pathology that is among the most challenging pathologies in the current medical scenario. SCA has a wide spectrum of clinical presentations, including degenerative and progressive forms with a decline in functional capacity, life-threatening characteristics, and long-standing presentation with great variability in symptoms and clinical severity. Our book addresses the background concepts, generalities, and particularities related to SCA, including clinical, neurological, genetic, and functional aspects. The book also gathers necessary information for functional classification, new approaches to medical and non-medical treatment, and rehabilitation/support, including aspects of palliative supportive care.

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Section: Ipsc-derived Models For Cerebellar Ataxiasmentioning

confidence: 99%

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

2022

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Human Pluripotent Stem Cell-Derived Cerebellar Neurons: From Development to Modeling Cerebellar Ataxias

Deleanu¹

2022

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

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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.

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iPS Cells and Spinocerebellar Ataxia

Cited by 2 publications

References 94 publications

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

Human Pluripotent Stem Cell-Derived Cerebellar Neurons: From Development to Modeling Cerebellar Ataxias

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