Molecular pathways of oligodendrocyte apoptosis revealed by mutations in the proteolipid protein gene

  title={Molecular pathways of oligodendrocyte apoptosis revealed by mutations in the proteolipid protein gene},
  author={C. Southwood and A. Gow},
  journal={Microscopy Research and Technique},
  • C. Southwood, A. Gow
  • Published 2001
  • Biology, Medicine
  • Microscopy Research and Technique
  • A decade after the genetic link was established between mutations in the proteolipid protein gene and two leukodystrophies, Pelizaeus‐Merzbacher disease and spastic paraplegia, the molecular mechanisms underlying pathogenesis are beginning to come to light. Data from animal models of these diseases suggest that the absence of proteolipid protein gene products in the central nervous system confers a relatively mild phenotype while missense mutations in and duplications of this gene give rise to… CONTINUE READING
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