Axonal Swellings and Degeneration in Mice Lacking the Major Proteolipid of Myelin

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 widespr...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 1998-06, Vol.280 (5369), p.1610-1613
Main Authors: Griffiths, Ian, Klugmann, Matthias, Anderson, Thomas, Yool, Donald, Thomson, Christine, Schwab, Markus H., Schneider, Armin, Zimmermann, Frank, McCulloch, Mailise, Nadon, Nancy, Nave, Klaus-Armin
Format: Article
Language:English
Subjects:
Anatomy
Animals
Axonal Transport
Axons
Axons - physiology
Axons - ultrastructure
Biological and medical sciences
Cell Communication
Central nervous system
Central Nervous System - ultrastructure
Classical genetics, quantitative genetics, hybrids
Female
Fundamental and applied biological sciences. Psychology
Genetics of eukaryotes. Biological and molecular evolution
Lipids
Mice
Mice, Neurologic Mutants
Models, Neurological
Motor Activity
Myelin
Myelin Proteolipid Protein - analysis
Myelin Proteolipid Protein - genetics
Myelin Proteolipid Protein - physiology
Myelin Sheath - chemistry
Myelin Sheath - physiology
Myelin Sheath - ultrastructure
Nerve Degeneration - pathology
Nerve Tissue Proteins
Nervous system
Neuroglia
Neurons
Oligodendroglia
Oligodendroglia - physiology
Optic nerve
Optic Nerve - ultrastructure
Optics
Organelles - ultrastructure
Spheroids
Spinal Cord - ultrastructure
Swelling
Transgenes
Vertebrata
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Summary: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.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.280.5369.1610