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

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.

• Authors: Fredrik I Gruenenfelder, Gemma Thomson, Jacques Penderis, Julia M Edgar
• Journal: Journal of anatomy (J Anat) Vol. 219 Issue 1 Pg. 33-43 (Jul 2011) ISSN: 1469-7580 [Electronic] England
• PMID: 21401588 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: © 2011 The Authors. Journal of Anatomy © 2011 Anatomical Society of Great Britain and Ireland.

Chemical References

• Myelin Proteolipid Protein

Topics

• Animals
• Axons (metabolism, physiology)
• Central Nervous System (metabolism, physiology)
• Disease Models, Animal
• Mice
• Myelin Proteolipid Protein (metabolism)
• Myelin Sheath (metabolism)
• Neuroglia (metabolism, physiology)
• Pelizaeus-Merzbacher Disease (metabolism, physiopathology)