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Abstract #2567

MRI Characterization of Cerebellar Atrophy in a Non-Human Primate Model of Neuronal Ceroid Lipofuscinosis

Ian Tagge1, Lois Colgin2, Rebecca Ducore2, Betsy Ferguson3, Steven Kohama4, Jodi McBride4, Martha Neuringer4, Sam Peterson4, Scott Wong5, Robert Zweig6, and Anne Lewis2

1Advanced Imaging Research Center, Oregon Health & Science University, Portland, OR, United States, 2Pathology, Oregon National Primate Research Center, 3Primate Genetics, Oregon National Primate Research Center, 4Oregon National Primate Research Center, 5VGTI, Oregon National Primate Research Center, 6Clinical Medicine, Oregon National Primate Research Center

Neuronal ceroid lipofuscinosis (NCL; also known as Batten Disease) is a fatal neurodegenerative disorder that typically presents in childhood. Currently, no treatments are known that can halt or reverse the effects of NCL. A naturally occurring form of NCL analogous to late infantile-onset NCL in humans has been identified in a population of Japanese macaques (JMs). MRI examinations revealed marked cerebellar degeneration in NCL animals >4y/o (~14y/o equivalent human age) compared with controls, which is strikingly similar to human disease. This novel JM model presents a new opportunity for characterizing disease progression, identifying biomarkers, and pre-clinical therapeutic testing.

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