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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

The fine structure of the cervical spinal cord, ventral root and brachial nerves in the wobbler (wr) mouse.

Much indirect evidence supports the prevailing concept that the wobbler mouse represents an inherited form of lower motor neuron degeneration, with resultant neurogenic muscular atrophy, due to an autosomal recessive gene (wr). This report, which emphasizes the fine structural changes in the wobbler mouse cervical spinal cord, ventral root and brachial nerves, both extends and modifies previously published data concerning the ultrastructural changes in this disorder. In addition to the more obvious neuronal cytoplasmic vacuolization known to occur, studies extended to a broader age range of wobbler mice have revealed neuronal alterations such as nonvacuolar dissolution of granular endoplasmic reticulum, proliferation of cytoplasmic microtubules and neurofilaments, proliferation of branched tubules resembling smooth endoplasmic reticulum, development of lipid droplets, and increased numbers of pleomorphic dense and lamellar cytoplasmic bodies. The spectrum of pathological changes in this murine form of motor neuron disease thus appears more complex and varied than has hitherto been appreciated, and may be relevant to the formulation of pathogenetic hypotheses.[1]


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