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Updated: Apr 30, 2026

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
Published on: October 21, 2014
Globoid cell leukodystrophy (Krabbe’s disease) in a Japanese domestic cat
Abstract:
A male Japanese domestic cat developed progressive limb paralysis from 4 months of age. The cat showed visual disorder, trismus and cognitive impairment and died at 9 months of age. At necropsy, significant discoloration of the white matter was observed throughout the brain and spinal cord. Histologically, severe myelin loss and gliosis were observed, especially in the internal capsule and cerebellum.In the lesions, severe infiltration of macrophages with broad cytoplasm filled with PAS-positive and nonmetachromatic granules (globoid cells) was evident. On the basis of these findings, the case was diagnosed as feline globoid cell leukodystrophy (Krabbe’s disease). Immunohistochemical observation indicated the involvement of oxidative stress and small HSP in the disease.
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