The English setter with ceroid-lipofuscinosis: a suitable model for the juvenile type of ceroid-lipofuscinosis in humans.

@article{Koppang1988TheES,
  title={The English setter with ceroid-lipofuscinosis: a suitable model for the juvenile type of ceroid-lipofuscinosis in humans.},
  author={Nils Koppang},
  journal={American journal of medical genetics. Supplement},
  year={1988},
  volume={5},
  pages={117-25}
}
  • Nils Koppang
  • Published 1988 in American journal of medical genetics. Supplement
Genetic and histological examinations (light and EM) of tissues of an inbred line of English setters have proved that these dogs suffer a general metabolic autosomal recessive disease, canine ceroid-lipofuscinosis (CCL) almost identical to the human Stengel-Batten-Spielmeyer-Vogt disease, or neuronal ceroid-lipofuscinosis (NCL). A controlled longitudinal morphologic study showed that the formation and accumulation of an autofluorescent lipopigment, identified as "ceroid" in the isolated state… CONTINUE READING

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