Abstract
Central nervous system myelin is formed from the lipid-rich, highly specialized oligoden-drocyte plasma membrane, which ensheaths axons, leaving spaces only at the nodes of Ranvier. Within the myelin sheath are many myelin-associated lipophilic proteins, the most abundant of which are myelin proteolipid protein (PLP) and myelin basic protein (MBP). These two proteins constitute 60–90% of myelin protein in mammalian species.1
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References
Lees, M.B., Brostoff, S.W., 1984, Proteins of myelin: in Morell P (ed): Myelin, New York, Plenum Press, 1984, pp. 197–224.
Unwin, N., 1989, The structure of ion channels in membranes of excitable cells. Neuron 3: 3665–676.
Macklin, W.B., Oberfeld, E., Lees, M.B., 1983, Electroblot analysis of rat myelin proteolipid protein and basic protein during development. Dev. Neurosci. 6: 161–168.
Garbern, J., Cambi, F., Shy, M., and Kamholtz, J., 1999, The molecular pathogenesis of Pelizaeus-Merzbacher disease, Arch. Neurol. 56: 1210–1214.
Renier W.O., Gabreels F.J.M., Hustinx T.W.J., Jaspar H.H.J., Geleen J.A.G., Van Haelst U.J.G., Lommen E.J.P., Ter Haar, B.G.A., 1981, Connatal Pelizaeus-Merzbacher Disease with Congenital Stridor in Two Maternal Counsins. Acta. Neuropathol. (Berl) 54: 11–17.
Knapp, P.E., 1996, Proteolipid protein: is it more than just a structural component of Myelin? Devel. Neurosci. 18: 297–308.
Boison, D., and Stoffel, W., 1989, A point mutation in exon3 (A-C, Thr 75-Pro) of the myelin proteolipid protein causes dysmyelination and oligodendrocyte death. EMBO J 8: 3295–3302.
Miller, M.J., Haxhiu, M.A., Gudz, T.L., Kangas, C., and Macklin, W.B., 2003, Proteolipd protein gene mutation induces altered ventilatory response to hypoxia in the myelin deficient rat. J. Neurosci., 23, 2265–2273.
Mallon, B.S., Shick, H.E., Kidd, G.J. and Macklin, W.B., 2002, Proteolipid promotor activity distinguishes two populations of NG2-positive cells throughout neonatal cortical development. J. Neurosci. 22(3): 876–885
Ang, R.C., Hoop, B. and Kazemi, H., 1992, Role of glutamate as the central neurotransmitter in the hypoxic ventilatory response, J. Applied Physiol. 72: 1480–1487.
Ohtake, P.J., Simakajornboon, N., Fehniger, M.D., Xue, Y. and Gozal, D., 2000, N-methyl-D-aspartate receptor expression in the nucleus tractus solitarii and maturation of hypoxic ventilatory response in the rat, Am. J. Resp Crit. Care Med., 162: 1140–1147.
Ohtake, P.J., Torres, J.E., Gozal, Y.M., Graff, G.R. and Gozal, D., 1998, NMDA receptors mediate peripheral chemoreceptor afferent input in the conscious rat, J. Appl. Physiol. 84: 853–861
Mullen, R.J., Buck, C.R. and Smith, A., 1992, NeuN, a neuronal specific nuclear protein in vertebrates, Devel 116: 201–211.
Gow, A., Friedrich, V.L. and Lazzarini, R.A., 1994, Many natural occurring mutations of myelin proteolipid protein impair its intracellular transport, J. Neurosci. Res 15: 383–394.
Roach, A., Takahashi, N., Pratcheve D., Ruddle, F., Hood, L., 1985, Chromosomal mapping of mouse myelin basic protein gene and structure and transcription of the partially deleted gene in shiverer mutant mice, Cell 42: 149–155.
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Miller, M.J., Haxhiu, M.A., Kangas, C.D., Georgiadis, P., Gudz, T.I., Macklin, W.B. (2004). Selective Alteration of the Ventilatory Response to Hypoxia Results from Mutation in the Myelin Proteolipid Protein Gene. In: Champagnat, J., Denavit-Saubié, M., Fortin, G., Foutz, A.S., Thoby-Brisson, M. (eds) Post-Genomic Perspectives in Modeling and Control of Breathing. Advances in Experimental Medicine and Biology, vol 551. Springer, Boston, MA. https://doi.org/10.1007/0-387-27023-X_14
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DOI: https://doi.org/10.1007/0-387-27023-X_14
Publisher Name: Springer, Boston, MA
Print ISBN: 978-0-306-48507-7
Online ISBN: 978-0-387-27023-4
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