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English setter model and juvenile ceroid-lipofuscinosis in man
Authors:Nils Koppang
Abstract:The etiology of the juvenile type of the human ceroid-lipofuscinosis (JCL) is unknown, in spite of the fact that the first report of this disease was given more than 160 years ago. The necessity of good animal models for scientific progress in chronic metabolic diseases in humans is obvious. The inbred strain of English setter with ceroid-lipofuscinosis (CCL) seems to be a perfect model for human JCL. Dogs with CCL and organs for research purposes are available from Dr. Koppang's experimental kennel in Norway.
Keywords:amaurotic familial idiocy  Batten disease  Neuronal ceroid-lipofuscinosis  canine ceroid-lipofuscinosis (CCL)  electroencephalogram  retinal pigment epithelium  rod outer segment  standing potential  dog model for juvenile ceroid-lipofuscinosis
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