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August 1967

The Wabbler-Lethal Mouse: An Electron Microscopic Study of the Nervous System

Author Affiliations

St Louis
From the departments of anatomy and pathology, and the Beaumont-May Institute of Neurology, Washington University School of Medicine, St Louis. Dr. Chenard's present address is 1301 Pelletier St, Quebec 6, Canada.

Arch Neurol. 1967;17(2):153-161. doi:10.1001/archneur.1967.00470260043004

GENETICALLY determined neurologic variants in inbred mouse colonies are of great interest because they may provide clues to the nature of inherited neurologic diseases in man. The wabbler-lethal (wlwl) mouse, a mutation in the colony at the Roscoe B. Jackson Memorial Laboratory,1-3 develops neurologic signs early and rarely survives more than a month. Marchi stains reveal myelin involvement in the brain stem and spinal cord with minimal involvement of the cerebrum limited to the internal capsule. Harman2,4 showed that changes in myelin, already evident at seven days, become increasingly severe during the short life span of affected animals. Similar Marchipositive material was not evident in control material and was only scattered and sparse in littermate heterozygotes.

Harman4 considered the wabbler-lethal mouse to have a genetically controlled defect in myelinization. No chemical abnormality was detectable, as is also true in early stages for wallerian degeneration when

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