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CSPα-deficiency causes massive and rapid photoreceptor degeneration

Opened Access CSPα-deficiency causes massive and rapid photoreceptor degeneration

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Autor: Schmitz, Frank
Tabares Domínguez, Lucía
Khimich, Darina
Strenzke, Nicola
Villa Polo, Pedro de la
Castellano Muñoz, Manuel
Bulankina, Anna
Moser, Tobias
Fernández-Chacón, Rafael
Südhof, Thomas C.
Departamento: Universidad de Sevilla. Departamento de Fisiología Médica y Biofísica
Fecha: 2006
Publicado en: Proceedings of the National Academy of Sciences of the United States of America, 103 (8), 2926-2931.
Tipo de documento: Artículo
Resumen: Cysteine string protein (CSP) α is an abundant synaptic vesicle protein that contains a DNA-J domain characteristic of Hsp40-type cochaperones. Previous studies showed that deletion of CSPα in mice leads to massive lethal neurodegeneration but did not clarify how the neurodegeneration affects specific subpopulations of neurons. Here, we analyzed the effects of the CSPα deficiency on tonically active ribbon synapses of the retina and the inner ear. We show that CSPα-deficient photoreceptor terminals undergo dramatic and rapidly progressive neurodegeneration that starts before eye opening and initially does not affect other retinal synapses. These changes are associated with progressive blindness. In contrast, ribbon synapses of auditory hair cells did not exhibit presynaptic impairments in CSPα-deficient mice. Hair cells, but not photoreceptor cells or central neurons, express CSPβ, thereby accounting for the lack of a hair-cell phenotype in CSPα knockout mice. Our data demonstrate tha...
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Cita: Schmitz, F., Tabares Domínguez, L., Khimich, D., Strenzke, N., Villa Polo, P.d.l., Castellano Muñoz, M.,...,Südhof, T.C. (2006). CSPα-deficiency causes massive and rapid photoreceptor degeneration. Proceedings of the National Academy of Sciences of the United States of America, 103 (8), 2926-2931.
Tamaño: 835.4Kb
Formato: PDF

URI: https://hdl.handle.net/11441/69842

DOI: 10.1073/pnas.0510060103

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