Article
Age-related axonal and myelin changes in the rumpshaker mutation of the Plp gene.
Acta neuropathologica - 1 Apr 2004
Edgar J M, McLaughlin M, Barrie J A, McCulloch M C, Garbern J, Griffiths I R
Abstract excerpt
The PLP1/Plp gene encodes proteolipid protein (PLP) and DM20, the major central nervous system myelin proteins. Mutations in the PLP1/ Plp gene cause dysmyelinating disorders in man and animals. The rumpshaker mutation was first identified in mice and later linked to a family diagnosed with neurological deficits akin to spastic paraplegia. The dysmyelination in the young rumpshaker mouse is well characterised....
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