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dc.contributor.author
Gonzalez Deniselle, Maria Claudia
dc.contributor.author
López Costa, Juan Jose
dc.contributor.author
Pecci Saavedra, Jorge
dc.contributor.author
Pietranera, Luciana
dc.contributor.author
Gonzalez, Susana Laura
dc.contributor.author
Garay, Laura Ines
dc.contributor.author
Guennoun, Rachida
dc.contributor.author
Schumacher, Michael
dc.contributor.author
de Nicola, Alejandro Federico
dc.date.available
2018-02-11T22:34:30Z
dc.date.issued
2002
dc.identifier.citation
Gonzalez Deniselle, Maria Claudia; López Costa, Juan Jose; Pecci Saavedra, Jorge; Pietranera, Luciana; Gonzalez, Susana Laura; et al.; Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease; Elsevier Science; Neurobiology of Disease; 11; 3; 2002; 457-468
dc.identifier.issn
0969-9961
dc.identifier.uri
http://hdl.handle.net/11336/36412
dc.description.abstract
Motor neuron degeneration characterizes the spinal cord of patients with amyotrophic lateral sclerosis and the Wobbler mouse mutant. Considering that progesterone (PROG) provides neuroprotection in experimental ischemia and injury, its potential role in neurodegeneration was studied in the murine model. Two-month-old symptomatic Wobbler mice were left untreated or received sc a 20-mg PROG implant for 15 days. Both light and electron microscopy of Wobbler mice spinal cord showed severely affected motor neurons with profuse cytoplasmic vacuolation of the endoplasmic reticulum and/or Golgi apparatus and ruptured mitochondria with damaged cristae, a profile indicative of a type II cytoplasmic form of cell death. In contrast to untreated mice, neuropathology was less severe in Wobbler mice receiving PROG; including a reduction of vacuolation and of the number of vacuolated cells and better conservation of the mitochondrial ultrastructure. In biochemical studies, we determined the mRNA for the α3 subunit of Na,K-ATPase, a neuronal enzyme controlling ion fluxes, neurotransmission, membrane potential, and nutrient uptake. In untreated Wobbler mice, mRNA levels in motor neurons were reduced by half compared to controls, whereas PROG treatment of Wobbler mice restored the expression of α3 subunit Na,K-ATPase mRNA. Therefore, PROG was able to rescue motor neurons from degeneration, based on recovery of histopathological abnormalities and of mRNA levels of the sodium pump. However, because the gene mutation in Wobbler mice is still unknown, further studies are needed to unveil the action of PROG and the mechanism of neuronal death in this genetic model of neurodegeneration.
dc.format
application/pdf
dc.language.iso
eng
dc.publisher
Elsevier Science
dc.rights
info:eu-repo/semantics/restrictedAccess
dc.rights.uri
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.subject
Neuroprotection
dc.subject
Spinal Cord
dc.subject
Progesterone
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Neurodegeneration
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Wobbler Mice
dc.subject
Amyotrophic Lateral Sclerosis
dc.subject.classification
Neurociencias
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Medicina Básica
dc.subject.classification
CIENCIAS MÉDICAS Y DE LA SALUD
dc.title
Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease
dc.type
info:eu-repo/semantics/article
dc.type
info:ar-repo/semantics/artículo
dc.type
info:eu-repo/semantics/publishedVersion
dc.date.updated
2017-12-28T17:49:44Z
dc.journal.volume
11
dc.journal.number
3
dc.journal.pagination
457-468
dc.journal.pais
Estados Unidos
dc.description.fil
Fil: Gonzalez Deniselle, Maria Claudia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Bioquímica Humana; Argentina
dc.description.fil
Fil: López Costa, Juan Jose. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Biología Celular y Neurociencia ; Argentina
dc.description.fil
Fil: Pecci Saavedra, Jorge. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Biología Celular y Neurociencia ; Argentina
dc.description.fil
Fil: Pietranera, Luciana. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Bioquímica Humana; Argentina
dc.description.fil
Fil: Gonzalez, Susana Laura. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Bioquímica Humana; Argentina
dc.description.fil
Fil: Garay, Laura Ines. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Bioquímica Humana; Argentina
dc.description.fil
Fil: Guennoun, Rachida. Inserm; Francia
dc.description.fil
Fil: Schumacher, Michael. Inserm; Francia
dc.description.fil
Fil: de Nicola, Alejandro Federico. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Bioquímica Humana; Argentina
dc.journal.title
Neurobiology of Disease
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S0969996102905644
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/doi/http://dx.doi.org/10.1006/nbdi.2002.0564


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