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Science 2 August 1985:
Vol. 229. no. 4712, pp. 470 - 472
DOI: 10.1126/science.3925555
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Articles

Science, Vol 229, Issue 4712, 470-472
Copyright © 1985 by American Association for the Advancement of Science

articles

Neurovisceral and skeletal GM1-gangliosidosis in dogs with beta-galactosidase deficiency

J Alroy, U Orgad, AA Ucci, SH Schelling, KL Schunk, CD Warren, SS Raghavan, and EH Kolodny

Beta-galactosidase-deficient siblings in two litters of English springer spaniel puppies showed a progressive neurological impairment, dwarfism, orbital hypertelorism, and dysostosis multiplex. An excess of GM1-ganglioside was found in the brain. Three abnormal oligosaccharides were present in samples of urine, brain, liver, and cartilage. Light microscopy of selected tissue specimens revealed cytoplasmic vacuoles in neurons, circulating blood cells, macrophages, and chondrocytes. Ultrastructural studies demonstrated that these membrane-bound vacuoles were of two types--one containing lamellated membranes and the other, finely granular material. These clinical and pathological findings are similar to those observed in human patients affected by the infantile form of GM1-gangliosidosis.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
GM1-gangliosidosis in Alaskan Huskies: Clinical and Pathologic Findings.
G. Muller, S. Alldinger, A. Moritz, A. Zurbriggen, N. Kirchhof, A. Sewell, and W. Baumgartner (2001)
Vet. Pathol. 38, 281-290
   Abstract »    Full Text »    PDF »
Biogenesis of Multilamellar Bodies via Autophagy.
M. Hariri, G. Millane, M.-P. Guimond, G. Guay, J. W. Dennis, and I. R. Nabi (2000)
Mol. Biol. Cell 11, 255-268
   Abstract »    Full Text »


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