Axonal swellings and degeneration in mice lacking the major proteolipid of myelin
Science, 1998•science.org
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 …
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 …
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.
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