Skip to main content

Selective Alteration of the Ventilatory Response to Hypoxia Results from Mutation in the Myelin Proteolipid Protein Gene

  • Conference paper
Post-Genomic Perspectives in Modeling and Control of Breathing

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

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Lees, M.B., Brostoff, S.W., 1984, Proteins of myelin: in Morell P (ed): Myelin, New York, Plenum Press, 1984, pp. 197–224.

    Google Scholar 

  2. Unwin, N., 1989, The structure of ion channels in membranes of excitable cells. Neuron 3: 3665–676.

    Article  Google Scholar 

  3. 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.

    Article  PubMed  Google Scholar 

  4. Garbern, J., Cambi, F., Shy, M., and Kamholtz, J., 1999, The molecular pathogenesis of Pelizaeus-Merzbacher disease, Arch. Neurol. 56: 1210–1214.

    Article  CAS  PubMed  Google Scholar 

  5. 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.

    Article  CAS  PubMed  Google Scholar 

  6. Knapp, P.E., 1996, Proteolipid protein: is it more than just a structural component of Myelin? Devel. Neurosci. 18: 297–308.

    Article  CAS  Google Scholar 

  7. 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.

    CAS  PubMed  Google Scholar 

  8. 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.

    CAS  PubMed  Google Scholar 

  9. 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

    CAS  PubMed  Google Scholar 

  10. 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.

    CAS  Google Scholar 

  11. 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.

    CAS  PubMed  Google Scholar 

  12. 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

    CAS  PubMed  Google Scholar 

  13. Mullen, R.J., Buck, C.R. and Smith, A., 1992, NeuN, a neuronal specific nuclear protein in vertebrates, Devel 116: 201–211.

    CAS  Google Scholar 

  14. 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.

    Google Scholar 

  15. 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.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2004 Kluwer Academic/Plenum Publishers, New York

About this paper

Cite this paper

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

Download citation

Publish with us

Policies and ethics