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Peripheral axotomy slows motoneuron degeneration in a transgenic mouse line expressing mutant SOD1 G93A
Author(s) -
Kong Jiming,
Xu Zuoshang
Publication year - 1999
Publication title -
journal of comparative neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.855
H-Index - 209
eISSN - 1096-9861
pISSN - 0021-9967
DOI - 10.1002/(sici)1096-9861(19990920)412:2<373::aid-cne13>3.0.co;2-n
Subject(s) - biology , axotomy , genetically modified mouse , sod1 , transgene , neuroscience , peripheral , microbiology and biotechnology , mutant , degeneration (medical) , genetics , gene , central nervous system , pathology , medicine
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that involves motoneuron degeneration, paralysis, and death. Mutations in Cu, Zn superoxide dismutase (SOD1) are one cause of this disease. It has been a puzzle as to why mutations in SOD1, an enzyme expressed in many neuronal types, selectively kill motoneurons. To begin to explore the factors that determine this selectivity, we carried out peripheral axotomy in mice expressing a mutant SOD1 (G93A mice) and controls (nontransgenic mice and mice expressing wild‐type human SOD1). Axotomy in controls induced a predicted axonal atrophy and a small degree of axon loss. The axonal atrophy led to an increase in the number of small axons and a decrease in the number of large axons. In contrast to the controls, axotomy in G93A mice reduced the extent of axon degeneration at the end stage of the disease, leading to an increase in the number of surviving motor axons. Interestingly, all of the increased surviving axons were in the axon group with diameters smaller than 4.5 μm. This result suggests an apparent threshold of vulnerability that is correlated with axon size. J. Comp. Neurol. 412:373–380, 1999. © 1999 Wiley‐Liss, Inc.

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