Please use this identifier to cite or link to this item: http://hdl.handle.net/10668/314
Title: Increased neurotransmitter release at the neuromuscular junction in a mouse model of polyglutamine disease.
Authors: Rozas, José L.
Gómez-Sánchez, Leonardo
Tomás-Zapico, Cristina
Lucas, José J
Fernández-Chacón, Rafael
metadata.dc.contributor.authoraffiliation: [Rozas,JL; Gómez-Sánchez,L;Fernández-Chacón,R]Instituto de Biomedicina de Sevilla. Hospital Universitario Virgen del Rocío. Consejo Superior de Investigaciones Científicas (CSIC.)Universidad de Sevilla, Departamento de Fisiología Médica y Biofísica Universidad de Sevilla, Sevilla, Spain. [Tomás-Zapico,C;Lucas,JJ] Centro de Biología Molecular Severo Ochoa, CSIC. Universidad Autónoma de Madrid, Madrid, Spain.[Rozas,JL;Gómez-Sánchez,L;Tomás-Zapico,C;Lucas,JJ;Fernández-Chacón,R]Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Spain.
metadata.dc.subject.mesh: Medical Subject Headings::Diseases::Nervous System Diseases::Neurodegenerative Diseases::Heredodegenerative Disorders, Nervous System::Huntington Disease
Medical Subject Headings::Phenomena and Processes::Cell Physiological Phenomena::Cell Physiological Processes::Signal Transduction::Synaptic Transmission
Issue Date: 10-Nov-2011
Publisher: Society for Neuroscience
Citation: Rozas JL, Gómez-Sánchez L, Tomás-Zapico C, Lucas JJ, Fernández-Chacón R. Increased neurotransmitter release at the neuromuscular junction in a mouse model of polyglutamine disease. J Neurosci. 2011 Jan 19;31(3):1106-13.
Abstract: In Huntington's disease (HD), the expansion of polyglutamine (polyQ) repeats at the N terminus of the ubiquitous protein huntingtin (htt) leads to neurodegeneration in specific brain areas. Neurons degenerating in HD develop synaptic dysfunctions. However, it is unknown whether mutant htt impacts synaptic function in general. To investigate that, we have focused on the nerve terminals of motor neurons that typically do not degenerate in HD. Here, we have studied synaptic transmission at the neuromuscular junction of transgenic mice expressing a mutant form of htt (R6/1 mice). We have found that the size and frequency of miniature endplate potentials are similar in R6/1 and control mice. In contrast, the amplitude of evoked endplate potentials in R6/1 mice is increased compared to controls. Consistent with a presynaptic increase of release probability, synaptic depression under high-frequency stimulation is higher in R6/1 mice. In addition, no changes were detected in the size and dynamics of the recycling synaptic vesicle pool. Moreover, we have found increased amounts of the synaptic vesicle proteins synaptobrevin 1,2/VAMP 1,2 and cysteine string protein-α, and the SNARE protein SNAP-25, concomitant with normal levels of other synaptic vesicle markers. Our results reveal that the transgenic expression of a mutant form of htt leads to an unexpected gain of synaptic function. That phenotype is likely not secondary to neurodegeneration and might be due to a primary deregulation in synaptic protein levels. Our findings could be relevant to understand synaptic toxic effects of proteins with abnormal polyQ repeats.
URI: http://hdl.handle.net/10668/314
metadata.dc.relation.publisherversion: http://www.jneurosci.org/content/31/3/1106.long#content-block
metadata.dc.identifier.doi: 10.15233/​JNEUROSCI.2011-10.201
ISSN: 1529-2401 (Online)
0270-6474 (Print)
Appears in Collections:01- Artículos - Hospital Virgen del Rocío
01- Artículos - IBIS. Instituto de Biomedicina de Sevilla

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