ArticleThe Journal of veterinary medical science2025
Assessment of motor deterioration in a cynomolgus macaque with neuronal ceroid lipofuscinosis type 2 disease using two behavioral analyses.
Article in The Journal of veterinary medical science, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
The classical late-infantile form of neuronal ceroid lipofuscinosis type 2 (CLN2 disease) is a fatal lysosomal disorder characterized by progressive motor decline and premature death. A CLN2 cynomolgus macaque (Macaca fascicularis) model closely replicates the late-stage clinical and pathological features of human CLN2. To evaluate motor deterioration in this model, we conducted two behavioral assessments-the CLN2 Macaque Clinical Rating Scale and the Apple test-from the pre-onset stage in one macaque. Hypokinesia was observed as the first symptom at 22 months of age, followed by a marked decline in forelimb movement from 27 months. These simple, non-invasive, and cost-effective tests are valuable tools for monitoring disease progression and may facilitate the future clinical evaluation of therapeutic strategies for CLN2.
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