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Axon–glial interaction in the CNS: what we have learned from mouse models of Pelizaeus–Merzbacher disease
Author(s) -
Gruenenfelder Fredrik I.,
Thomson Gemma,
Penderis Jacques,
Edgar Julia M.
Publication year - 2011
Publication title -
journal of anatomy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.932
H-Index - 118
eISSN - 1469-7580
pISSN - 0021-8782
DOI - 10.1111/j.1469-7580.2011.01363.x
Subject(s) - proteolipid protein 1 , myelin , myelin proteolipid protein , biology , neuroscience , axon , central nervous system , myelin basic protein
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.