Neuronal-Visceral GM 1 Gangliosidosis in a Dog with β-Galactosidase Deficiency
- 22 October 1976
- journal article
- research article
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 194 (4263) , 442-445
- https://doi.org/10.1126/science.824730
Abstract
A 9-month-old dog with a history of progressive motor dysfunction was shown to have a deficiency in brain β-galactosidase activity. The canine disease, like that of children with GM 1 gangliosidosis, is characterized by accumulation of GM 1 ganglioside in the brain, liver, and spleen, and membranous cytoplasmic bodies in neurons. The dog's pedigree suggests an autosomal recessive pattern of inheritance.This publication has 12 references indexed in Scilit:
- Composition and synthesis of gangliosides in mammary gland and milk of the bovineBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1974
- Studies on GM1-gangliosidosis, type IIActa Neuropathologica, 1974
- Inherited lysosomal storage diseases: an essay in comparative medicinePublished by Wiley ,1973
- Lipid composition of subcellular fractions from rat testisBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1972
- Generalized gangliosidosisThe Journal of Pediatrics, 1969
- [29] β-Galactosidase from rat brainPublished by Elsevier ,1969
- CHROMATOGRAPHlC SEPARATION OF HUMAN BRAIN GANGLIOSIDES*Journal of Neurochemistry, 1963
- A spectrophotometric determination of sphingosineJournal of Lipid Research, 1962
- The Thiobarbituric Acid Assay of Sialic AcidsJournal of Biological Chemistry, 1959
- DETERMINATION OF SERUM PROTEINS BY MEANS OF THE BIURET REACTIONJournal of Biological Chemistry, 1949