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The Journal of Neuroscience, April 14, 2004, 24(15):3890-3898; doi:10.1523/JNEUROSCI.4617-03.2004

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 Previous Article

Development/Plasticity/Repair
Dysmyelinated Lower Motor Neurons Retract and Regenerate Dysfunctional Synaptic Terminals

Xinghua Yin,¹ Grahame J. Kidd,¹ Erik P. Pioro,^1,2 Jennifer McDonough,¹ Ranjan Dutta,¹ M. Laura Feltri,³ Lawrence Wrabetz,³ Albee Messing,⁴ Ryan M. Wyatt,⁵ Rita J. Balice-Gordon,⁵ and Bruce D. Trapp¹

¹Department of Neurosciences, Lerner Research Institute, and ²Department of Neurology, Cleveland Clinic Foundation, Cleveland, Ohio 44195, ³San Raffaele Scientific Institute, Department of Biological and Technological Research, Milan 20132, Italy, ⁴Department of Pathobiological Sciences and Waisman Center, University of Madison-Wisconsin, Madison, Wisconsin 53705-2280, and ⁵Department of Neuroscience, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104

Axonal degeneration is the major cause of permanent neurological disability in individuals with inherited diseases of myelin. Axonal and neuronal changes that precede axonal degeneration, however, are not well characterized. We show here that dysmyelinated lower motor neurons retract and regenerate dysfunctional presynaptic terminals, leading to severe neurological disability before axonal degeneration. In addition, dysmyelination led to a decreased synaptic quantal content, an indicator of synaptic dysfunction. The amplitude and rise time of miniature endplate potentials were also increased, but these changes were primarily consistent with an increase in the passive membrane properties of the transgenic muscle fibers. Maintenance of synaptic connections should be considered as a therapeutic target for slowing progression of neurological disability in primary diseases of myelin.

Key words: myelin; axon; acetylcholine receptor; dysmyelination; axonal sprouting; neuromuscular junction

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Received Oct 13, 2003; revised February 27, 2004; accepted February 28, 2004.

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				R. H. Baloh, A. Strickland, E. Ryu, N. Le, T. Fahrner, M. Yang, R. Nagarajan, and J. Milbrandt Congenital Hypomyelinating Neuropathy with Lethal Conduction Failure in Mice Carrying the Egr2 I268N Mutation J. Neurosci., February 25, 2009; 29(8): 2312 - 2321. [Abstract] [Full Text] [PDF]

				J. Li, Y. Bai, K. Ghandour, P. Qin, M. Grandis, A. Trostinskaia, E. Ianakova, X. Wu, A. Schenone, J.-M. Vallat, et al. Skin biopsies in myelin-related neuropathies: bringing molecular pathology to the bedside Brain, May 1, 2005; 128(5): 1168 - 1177. [Abstract] [Full Text] [PDF]

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