Cerebellar Development and Circuit Maturation: A Common Framework for Spinocerebellar Ataxias.

Binda, Francesca; Pernaci, Carla; Saxena, Smita (2020). Cerebellar Development and Circuit Maturation: A Common Framework for Spinocerebellar Ataxias. Frontiers in neuroscience, 14(293), p. 293. Frontiers Research Foundation 10.3389/fnins.2020.00293

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Spinocerebellar ataxias (SCAs) affect the cerebellum and its afferent and efferent systems that degenerate during disease progression. In the cerebellum, Purkinje cells (PCs) are the most vulnerable and their prominent loss in the late phase of the pathology is the main characteristic of these neurodegenerative diseases. Despite the constant advancement in the discovery of affected molecules and cellular pathways, a comprehensive description of the events leading to the development of motor impairment and degeneration is still lacking. However, in the last years the possible causal role for altered cerebellar development and neuronal circuit wiring in SCAs has been emerging. Not only wiring and synaptic transmission deficits are a common trait of SCAs, but also preventing the expression of the mutant protein during cerebellar development seems to exert a protective role. By discussing this tight relationship between cerebellar development and SCAs, in this review, we aim to highlight the importance of cerebellar circuitry for the investigation of SCAs.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DCR Unit Sahli Building > Forschungsgruppe Neurologie
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR)

Graduate School:

Graduate School for Cellular and Biomedical Sciences (GCB)

UniBE Contributor:

Binda, Francesca; Pernaci, Carla and Saxena, Smita

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1662-4548

Publisher:

Frontiers Research Foundation

Language:

English

Submitter:

Chantal Kottler

Date Deposited:

14 Jul 2020 16:35

Last Modified:

10 Mar 2021 21:20

Publisher DOI:

10.3389/fnins.2020.00293

PubMed ID:

32300292

Uncontrolled Keywords:

Purkinje cell degeneration cerebellar circuits cerebellum circuit maturation spinocerebellar ataxia

BORIS DOI:

10.7892/boris.145199

URI:

https://boris.unibe.ch/id/eprint/145199

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