Axon-glial interaction in the CNS: what we have learned from mouse models of Pelizaeus-Merzbacher disease

Gruenenfelder, F.I., Thomson, G., Penderis, J. and Edgar, J.M. (2011) Axon-glial interaction in the CNS: what we have learned from mouse models of Pelizaeus-Merzbacher disease. Journal of Anatomy, 219(1), pp. 33-43. (doi: 10.1111/j.1469-7580.2011.01363.x)

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Abstract

In the central nervous system (CNS) the majority of axons are surrounded by a myelin sheath, which is produced by oligodendrocytes. Myelin is a lipid-rich insulating material that facilitates the rapid conduction of electrical impulses along the myelinated nerve fibre. Proteolipid protein and its isoform DM20 constitute the most abundant protein component of CNS myelin. Mutations in the PLP1 gene encoding these myelin proteins cause Pelizaeus–Merzbacher disease and the related allelic disorder, spastic paraplegia type 2. Animal models of these diseases, particularly models lacking or overexpressing Plp1, have shed light on the interplay between axons and oligodendrocytes, and how one component influences the other.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Penderis, Professor Jacques and Thomson, Miss Gemma and Edgar, Professor Julia
Authors: Gruenenfelder, F.I., Thomson, G., Penderis, J., and Edgar, J.M.
Subjects:Q Science > QM Human anatomy
College/School:College of Medical Veterinary and Life Sciences > School of Infection & Immunity
College of Medical Veterinary and Life Sciences > School of Biodiversity, One Health & Veterinary Medicine
Journal Name:Journal of Anatomy
ISSN:0021-8782
Published Online:14 March 2011

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