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pubmed:abstractText |
Juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease, is a pediatric neurodegenerative disease characterized by vision loss, seizure activity, cognitive decline, and premature death. Discovery of the Batten disease-related gene, CLN3, led to creation of a Cln3 protein-deficient mouse model (Cln3-/-), which recapitulates some of the histopathologic characteristics of the human condition. We hypothesized that lack of Cln3 would alter seizure-related behavioral parameters.
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pubmed:affiliation |
Center for Aging and Developmental Biology, Department of Biochemistry and Biophysics, and Department of Neurology, University of Rochester School of Medicine and Dentistry, Rochester, New York 14642, USA.
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