Animal model: ceroidosis (ceroid-lipofuscinosis) in Australian cattle dogs.
Ceroid-lipofuscinosis is described in Australian Cattle dogs. Lesions included storage of ceroid-lipofuscin in most tissues with characteristic ultrastructural inclusion body patterns in neurons and other cells. Dolichol concentration of the affected dog's brain was similar to those in age-matched control dog brains. However, concentrations in brain, liver and kidneys were markedly higher than in a slightly younger dog. Biochemical data including lysosomal enzyme activities exclude other lysosomal storage diseases. The clinical and pathological features of this disorder resemble those of the juvenile Spielmeyer-Vogt type of Batten disease in humans.
D000367:Age Factors; D000818:Animals; D002540:Cerebral Cortex / Q000378:metabolism; D004195:Disease Models, Animal; D004283:Dog Diseases / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D004285:Dogs; D004286:Dolichols / Q000378:metabolism; D005808:Genes, Recessive; D050356:Lipid Metabolism; D008247:Lysosomes / Q000201:enzymology; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D010375:Pedigree
D004286:Dolichols