Article
Transgenic and natural mouse models of proteolipid protein (PLP)-related dysmyelination and demyelination.
Brain pathology (Zurich, Switzerland) - 1 Jul 1995
Griffiths I R, Schneider A, Anderson J, Nave K A
Abstract excerpt
The X chromosome-linked PLP/DM-20 gene is the CNS myelin gene most frequently associated with mutations, resulting in dysmyelination in several species including man (Pelizaeus-Merzbacher disease, X-linked Spastic Paraplegia). The pathology of most PLP gene mutations is characterized by hypomyelination, glial cell proliferation, increased numbers of microglia, and premature oligodendrocyte death. In most mutants,...
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