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CSPα, a molecular co-chaperone essential for short and long-term synaptic maintenance

Opened Access CSPα, a molecular co-chaperone essential for short and long-term synaptic maintenance

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Autor: López Ortega, Elena
Ruíz Laza, Rocío
Tabares Domínguez, Lucía
Departamento: Universidad de Sevilla. Departamento de Bioquímica y Biología Molecular
Fecha: 2017-02-10
Publicado en: Frontiers in Neuroscience, 11 (FEB), 39-.
Tipo de documento: Artículo
Resumen: Cysteine string protein a (CSPα) is a vesicle protein located in the presynaptic terminal of most synapses. CSPα is an essential molecular co-chaperone that facilitates the correct folding of proteins and the assembly of the exocytic machinery. The absence of this protein leads to altered neurotransmitter release and neurodegeneration in multiple model systems, from flies to mice. In humans, CSPα mutations are associated with the development of neuronal ceroid lipofuscinosis (NCL), a neurodegenerative disease characterized by intracellular accumulation of lysosomal material. Here, we review the physiological role of CSPα and the pathology resulting from the homozygous deletion of the gene or its mutations. In addition, we investigate whether long-term moderate reduction of the protein produces motor dysfunction. We found that 1-year-old CSPα heterozygous mice display a reduced ability to sustain motor unit recruitment during repetitive stimulation, which indicates that physiological l...
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Cita: López Ortega, E., Ruíz Laza, R. y Tabares Domínguez, L. (2017). CSPα, a molecular co-chaperone essential for short and long-term synaptic maintenance. Frontiers in Neuroscience, 11 (FEB), 39-.
Tamaño: 990.1Kb
Formato: PDF

URI: http://hdl.handle.net/11441/62600

DOI: 10.3389/fnins.2017.00039

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