Proteolipid protein is necessary in peripheral as well as central myelin

James Y. Garbern, Franca Cambi, Xue Ming Tang, Anders A F Sima, Jean Michel Vallat, E. Peter Bosch, Richard Lewis, Michael Shy, Jasloveleen Sohi, George Kraft, Ke Lian Chen, Indira Joshi, Debra G B Leonard, William Johnson, Wendy Raskind, Stephen Dlouhy, Victoria Pratt, M. Edward Hodes, Thomas Bird, John Kamholz

Research output: Contribution to journalArticle

113 Citations (Scopus)

Abstract

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 normal peripheral nerve demonstrates that PLP is localized to compact myelin. This and the clinical and pathologic observations of the PLP null phenotype indicate that PLP/DM20 is necessary for proper myelin function both in the central and peripheral nervous systems.

Original languageEnglish
Pages (from-to)205-218
Number of pages14
JournalNeuron
Volume19
Issue number1
DOIs
StatePublished - Jul 1997

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Proteolipids
Myelin Sheath
Proteins
Mutation
Central Nervous System
Phenotype
Schwann Cells
Peripheral Nervous System
Peripheral Nervous System Diseases
Peripheral Nerves
Alleles

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Garbern, J. Y., Cambi, F., Tang, X. M., Sima, A. A. F., Vallat, J. M., Bosch, E. P., ... Kamholz, J. (1997). Proteolipid protein is necessary in peripheral as well as central myelin. Neuron, 19(1), 205-218. https://doi.org/10.1016/S0896-6273(00)80360-8

Proteolipid protein is necessary in peripheral as well as central myelin. / Garbern, James Y.; Cambi, Franca; Tang, Xue Ming; Sima, Anders A F; Vallat, Jean Michel; Bosch, E. Peter; Lewis, Richard; Shy, Michael; Sohi, Jasloveleen; Kraft, George; Chen, Ke Lian; Joshi, Indira; Leonard, Debra G B; Johnson, William; Raskind, Wendy; Dlouhy, Stephen; Pratt, Victoria; Hodes, M. Edward; Bird, Thomas; Kamholz, John.

In: Neuron, Vol. 19, No. 1, 07.1997, p. 205-218.

Research output: Contribution to journalArticle

Garbern, JY, Cambi, F, Tang, XM, Sima, AAF, Vallat, JM, Bosch, EP, Lewis, R, Shy, M, Sohi, J, Kraft, G, Chen, KL, Joshi, I, Leonard, DGB, Johnson, W, Raskind, W, Dlouhy, S, Pratt, V, Hodes, ME, Bird, T & Kamholz, J 1997, 'Proteolipid protein is necessary in peripheral as well as central myelin', Neuron, vol. 19, no. 1, pp. 205-218. https://doi.org/10.1016/S0896-6273(00)80360-8
Garbern JY, Cambi F, Tang XM, Sima AAF, Vallat JM, Bosch EP et al. Proteolipid protein is necessary in peripheral as well as central myelin. Neuron. 1997 Jul;19(1):205-218. https://doi.org/10.1016/S0896-6273(00)80360-8
Garbern, James Y. ; Cambi, Franca ; Tang, Xue Ming ; Sima, Anders A F ; Vallat, Jean Michel ; Bosch, E. Peter ; Lewis, Richard ; Shy, Michael ; Sohi, Jasloveleen ; Kraft, George ; Chen, Ke Lian ; Joshi, Indira ; Leonard, Debra G B ; Johnson, William ; Raskind, Wendy ; Dlouhy, Stephen ; Pratt, Victoria ; Hodes, M. Edward ; Bird, Thomas ; Kamholz, John. / Proteolipid protein is necessary in peripheral as well as central myelin. In: Neuron. 1997 ; Vol. 19, No. 1. pp. 205-218.
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