Proteolipid Protein Is Necessary in Peripheral as Well as Central Myelin

  title={Proteolipid Protein Is Necessary in Peripheral as Well as Central Myelin},
  author={James Y. Garbern and Franca Cambi and Xue-ming Tang and Anders A.F Sima and Jean Michel Vallat and E Bosch and Richard Lewis and Michael Shy and Jasloveleen Sohi and George Kraft and Ke Chen and Indira D. Joshi and Debra G.B Leonard and William Johnson and Wendy Raskind and Stephen R. Dlouhy and Victoria M. Pratt and M Z Hodes and Thomas T Bird and John A Kamholz},
Alternative products of the proteolipid protein gene (PLP), proteolipid protein (PLP) and DM20, are major components of compact myelin in the central nervous system, but quantitatively minor constituents of Schwann cells. A family with a null allele of PLP has a less severe CNS phenotype than those with other types of PLP mutations. Moreover, individuals with PLP null mutations have a demyelinating peripheral neuropathy, not seen with other PLP mutations of humans or animals. Direct analysis of… CONTINUE READING
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