Axonal swellings and degeneration in mice lacking the major proteolipid of myelin

I R Griffiths, M Klugmann, T. Anderson, Donald Yool, Christine Thomson, M H Schwab, A Schneider, F Zimmerman, M McCulloch, Klaus-Armin Nave

Research output: Contribution to journalArticlepeer-review

Abstract

Glial cells produce myelin and contribute to axonal morphology in the nervous system. Two myelin membrane proteolipids, PLP and DM20, were shown to be essential for the integrity of myelinated axons. In the absence of PLP-DM20, mice assembled compact myelin sheaths but subsequently developed widespread axonal swellings and degeneration, associated predominantly with small-caliber nerve fibers. Similar swellings were absent in dysmyelinated shiverer mice, which lack myelin basic protein (MBP), but recurred in MBP*PLP double mutants. Thus, fiber degeneration, which was probably secondary to impaired axonal transport, could indicate that myelinated axons require local oligodendroglial support.
Original languageEnglish
Pages (from-to)1610-3
JournalScience
Volume280
Issue number5369
Publication statusPublished - 5 Jun 1998

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