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      A lysosomal storage disease of Romney sheep that resembles human type 3 GM1 gangliosidosis.

      1 ,

      Acta neuropathologica

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          Abstract

          During a large neuropathological survey of clinically normal sheep for evidence of scrapie, three adult Romney ewes from the same farm were found to have populations of distended neurons containing granular eosinophilic storage material. The affected neurons were confined to the striatum. The granules had the tinctorial properties of glycolipid and the ultrastructural appearance of spherical bodies containing concentric membranous whorls. The bodies resembled those of GM1 gangliosidosis. The restricted neuroanatomical distribution of changes together with the age of the sheep suggests a similarity to human type 3 GM1 gangliosidosis, which has not previously been reported in animals.

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          Author and article information

          Journal
          Acta Neuropathol.
          Acta neuropathologica
          0001-6322
          0001-6322
          Mar 2001
          : 101
          : 3
          Affiliations
          [1 ] Neuropathology Unit, Veterinary Laboratories Agency-Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK. s.ryder@vla.maff.gov.uk
          Article
          11307621
          2353aa3e-b5b8-4cb0-9c2f-0c86b6177570

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