Artículo

Gonzalez Deniselle, M.C.; López-Costa, J.J.; Saavedra, J.P.; Pietranera, L.; Gonzalez, S.L.; Garay, L.; Guennoun, R.; Schumacher, M.; De Nicola, A.F. "Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease" (2002) Neurobiology of Disease. 11(3):457-468
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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. © 2003 Elsevier Science (USA).

Registro:

Documento: Artículo
Título:Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease
Autor:Gonzalez Deniselle, M.C.; López-Costa, J.J.; Saavedra, J.P.; Pietranera, L.; Gonzalez, S.L.; Garay, L.; Guennoun, R.; Schumacher, M.; De Nicola, A.F.
Filiación:Inst. de Biol. y Med. Experimental, Departamento de Bioquimica Humana, Universidad de Buenos Aires, 1428 Buenos Aires, Argentina
Inst. Biol. Cel. Neurociencias P., Facultad de Medicina, Universidad de Buenos Aires, 1121 Buenos Aires, Argentina
INSERM U488, Kremlin-Bicêtre, France
Inst. de Biol. y Med. Experimental, Obligado 2490, 1428 Buenos Aires, Argentina
Palabras clave:Amyotrophic lateral sclerosis; Neurodegeneration; Neuroprotection; Progesterone; Spinal cord; Wobbler mice; adenosine triphosphatase (potassium sodium); adenosine triphosphatase (potassium); messenger RNA; progesterone; alpha chain; animal experiment; animal model; animal tissue; article; brain mitochondrion; cell vacuole; controlled study; cytoplasm; disease model; electron microscopy; endoplasmic reticulum; female; gene mutation; Golgi complex; ion current; male; membrane potential; motor neuron disease; mouse; nerve cell necrosis; nerve degeneration; neuropathology; neuroprotection; neurotransmission; nonhuman; priority journal; protein expression; spinal cord motoneuron
Año:2002
Volumen:11
Número:3
Página de inicio:457
Página de fin:468
DOI: http://dx.doi.org/10.1006/nbdi.2002.0564
Título revista:Neurobiology of Disease
Título revista abreviado:Neurobiol. Dis.
ISSN:09699961
CODEN:NUDIE
CAS:adenosine triphosphatase (potassium sodium); progesterone, 57-83-0
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_09699961_v11_n3_p457_GonzalezDeniselle

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Citas:

---------- APA ----------
Gonzalez Deniselle, M.C., López-Costa, J.J., Saavedra, J.P., Pietranera, L., Gonzalez, S.L., Garay, L., Guennoun, R.,..., De Nicola, A.F. (2002) . Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease. Neurobiology of Disease, 11(3), 457-468.
http://dx.doi.org/10.1006/nbdi.2002.0564
---------- CHICAGO ----------
Gonzalez Deniselle, M.C., López-Costa, J.J., Saavedra, J.P., Pietranera, L., Gonzalez, S.L., Garay, L., et al. "Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease" . Neurobiology of Disease 11, no. 3 (2002) : 457-468.
http://dx.doi.org/10.1006/nbdi.2002.0564
---------- MLA ----------
Gonzalez Deniselle, M.C., López-Costa, J.J., Saavedra, J.P., Pietranera, L., Gonzalez, S.L., Garay, L., et al. "Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease" . Neurobiology of Disease, vol. 11, no. 3, 2002, pp. 457-468.
http://dx.doi.org/10.1006/nbdi.2002.0564
---------- VANCOUVER ----------
Gonzalez Deniselle, M.C., López-Costa, J.J., Saavedra, J.P., Pietranera, L., Gonzalez, S.L., Garay, L., et al. Progesterone neuroprotection in the Wobbler mouse, a genetic model of spinal cord motor neuron disease. Neurobiol. Dis. 2002;11(3):457-468.
http://dx.doi.org/10.1006/nbdi.2002.0564