title: 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.affiliation: Applied Neurobiology Group, Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, University of Glasgow, Scotland, UK.
authors.forename: Fredrik I
authors.initials: FI
authors.lastname: Gruenenfelder
authors.forename: Gemma
authors.initials: G
authors.lastname: Thomson
authors.forename: Jacques
authors.initials: J
authors.lastname: Penderis
authors.forename: Julia M
authors.initials: JM
authors.lastname: Edgar
mesh_terms: D000818:Animals; D001369:Axons / Q000378:metabolism / Q000502:physiology*; D002490:Central Nervous System / Q000378:metabolism / Q000502:physiology*; D004195:Disease Models, Animal; D051379:Mice; D018991:Myelin Proteolipid Protein / Q000378:metabolism; D009186:Myelin Sheath / Q000378:metabolism; D009457:Neuroglia / Q000378:metabolism / Q000502:physiology*; D020371:Pelizaeus-Merzbacher Disease / Q000378:metabolism / Q000503:physiopathology*
chemical_list: D018991:Myelin Proteolipid Protein