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Animal model: Ceroidosis (ceroid-lipofuscinosis) in Australian Cattle dogs
- Source :
- American Journal of Medical Genetics. 26:891-898
- Publication Year :
- 1987
- Publisher :
- Wiley, 1987.
-
Abstract
- 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.
- Subjects :
- congenital, hereditary, and neonatal diseases and abnormalities
Pathology
medicine.medical_specialty
Batten disease
Genes, Recessive
Biology
chemistry.chemical_compound
Dogs
Dolichol
Animal model
Neuronal Ceroid-Lipofuscinoses
Dolichols
Internal medicine
medicine
Animals
Juvenile
Dog Diseases
Pathological
Genetics (clinical)
Cerebral Cortex
Age Factors
nutritional and metabolic diseases
Lipid metabolism
Lipid Metabolism
medicine.disease
Pedigree
Disease Models, Animal
medicine.anatomical_structure
Endocrinology
chemistry
Cerebral cortex
Neuronal ceroid lipofuscinosis
Lysosomes
Subjects
Details
- ISSN :
- 10968628 and 01487299
- Volume :
- 26
- Database :
- OpenAIRE
- Journal :
- American Journal of Medical Genetics
- Accession number :
- edsair.doi.dedup.....da1a6503cd662a70ab527741994f4369