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 R. Dlouhy; Victoria Pratt; M. Edward Hodes; Thomas Bird; John Kamholz

doi:10.1016/S0896-6273(00)80360-8

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 R. Dlouhy, Victoria Pratt, M. Edward Hodes, Thomas Bird, John Kamholz

Neurology

Research output: Contribution to journal › Article › peer-review

116 Scopus citations

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 language	English (US)
Pages (from-to)	205-218
Number of pages	14
Journal	Neuron
Volume	19
Issue number	1
DOIs	https://doi.org/10.1016/S0896-6273(00)80360-8
State	Published - Jul 1997

ASJC Scopus subject areas

General Neuroscience

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Garbern, J. Y., Cambi, F., Tang, X. M., Sima, A. A. F., Vallat, J. M., Bosch, E. P., Lewis, R., Shy, M., Sohi, J., Kraft, G., Chen, K. L., Joshi, I., Leonard, D. G. B., Johnson, W., Raskind, W., Dlouhy, S. R., Pratt, V., Hodes, M. E., Bird, T., & 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

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, SR, 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

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title = "Proteolipid protein is necessary in peripheral as well as central myelin",

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.",

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

note = "Funding Information: M. S. and J. K. are supported by the Muscular Dystrophy Association and the Charcot-Marie-Tooth Association. M. E. H. is supported by the United Leukodystrophy Foundation. F. C. is a Clinical Investigator Development awardee, NS01726, from the NINDS. T.B. is a recipient of Veterans Administration research funds. The authors thank Drs. Joyce Benjamins, John Whittaker, and Wendy Macklin for the gifts of antisera. The authors are especially grateful to the families for their participation in this study. The authors gratefully acknowledge the help of C. Dolan and Dr. M. Dolan.",

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AU - Cambi, Franca

AU - Tang, Xue Ming

AU - Sima, Anders A.F.

AU - Vallat, Jean Michel

AU - Bosch, E. Peter

AU - Lewis, Richard

AU - Shy, Michael

AU - Sohi, Jasloveleen

AU - Kraft, George

AU - Chen, Ke Lian

AU - Joshi, Indira

AU - Leonard, Debra G.B.

AU - Johnson, William

AU - Raskind, Wendy

AU - Dlouhy, Stephen R.

AU - Pratt, Victoria

AU - Hodes, M. Edward

AU - Bird, Thomas

AU - Kamholz, John

N1 - Funding Information: M. S. and J. K. are supported by the Muscular Dystrophy Association and the Charcot-Marie-Tooth Association. M. E. H. is supported by the United Leukodystrophy Foundation. F. C. is a Clinical Investigator Development awardee, NS01726, from the NINDS. T.B. is a recipient of Veterans Administration research funds. The authors thank Drs. Joyce Benjamins, John Whittaker, and Wendy Macklin for the gifts of antisera. The authors are especially grateful to the families for their participation in this study. The authors gratefully acknowledge the help of C. Dolan and Dr. M. Dolan.

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AB - 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.

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Proteolipid protein is necessary in peripheral as well as central myelin

Abstract

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