English setter model and juvenile ceroid-lipofuscinosis in man

Am J Med Genet. 1992 Feb 15;42(4):599-604. doi: 10.1002/ajmg.1320420434.

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Ascorbic Acid / therapeutic use
  • Bone Marrow Transplantation
  • Brain / pathology
  • Butylated Hydroxytoluene / therapeutic use
  • Disease Models, Animal*
  • Dogs*
  • Electroencephalography
  • Female
  • Humans
  • Infant
  • Liver Transplantation
  • Male
  • Methionine / therapeutic use
  • Neuronal Ceroid-Lipofuscinoses* / drug therapy
  • Neuronal Ceroid-Lipofuscinoses* / pathology
  • Pigment Epithelium of Eye / pathology
  • Pigment Epithelium of Eye / ultrastructure
  • Vitamin E / therapeutic use

Substances

  • Vitamin E
  • Butylated Hydroxytoluene
  • Methionine
  • Ascorbic Acid