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The wobbler mouse mutation, an autosomal recessive mutation, leads to motoneuron degeneration in early post-natal development. Transgenic mice in which neurons overexpress human bcl2 transgene have been generated: the overexpression of bcl2 reduces the neuron loss during naturally occurring and experimentally-induced cell deaths. In the present study, we generate mice co-expressing the wobbler mutant...
Mice affected by the wobbler mutation are characterized by a muscular atrophy associated with motoneuron degeneration. As soon as the first clinical signs of the disease appear, reactive astrocytes, strongly glial fibrillary acidic protein (GFAP)-positive, are observed in the spinal cord grey matter. They become prevalent at all levels with disease progression. Immunostaining of glutamine synthetase...
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