{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T18:25:09Z","timestamp":1777919109969,"version":"3.51.4"},"reference-count":62,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2014,9,17]],"date-time":"2014-09-17T00:00:00Z","timestamp":1410912000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Vet Pathol"],"published-print":{"date-parts":[[2015,5]]},"abstract":"<jats:p>\n                    Clinical, gross, histopathologic, electron microscopic findings and enzymatic analysis of 4 captive, juvenile springboks ( Antidorcas marsupialis) showing both polycystic kidneys and a storage disease are described. Springbok offspring (4 of 34; 12%) were affected by either one or both disorders in a German zoo within a period of 5 years (2008\u20132013). Macroscopic findings included bilaterally severely enlarged kidneys displaying numerous cysts in 4 animals and superior brachygnathism in 2 animals. Histopathologically, kidneys of 4 animals displayed cystic dilation of the renal tubules. In addition, abundant cytoplasmic vacuoles with a diameter ranging from 2 to 10 \u03bcm in neurons of the central and peripheral nervous system, hepatocytes, thyroid follicular epithelial cells, pancreatic islets of Langerhans and renal tubular cells were found in 2 springbok neonates indicative of an additional storage disease. Ultrastructurally, round electron-lucent vacuoles, up to 4 \u03bcm in diameter, were present in neurons. Enzymatic analysis of liver and kidney tissue of 1 affected springbok revealed a reduced activity of total hexosaminidase (Hex) with relatively increased HexA activity at the same level of total Hex, suggesting a hexosaminidase defect. Pedigree analysis suggested a monogenic autosomal recessive inheritance for both diseases. In summary, related springboks showed 2 different changes resembling both polycystic kidney and a GM\n                    <jats:sub>2<\/jats:sub>\n                    gangliosidosis similar to the human Sandhoff disease. Whether the simultaneous occurrence of these 2 entities represents an incidental finding or has a genetic link needs to be investigated in future studies.\n                  <\/jats:p>","DOI":"10.1177\/0300985814549210","type":"journal-article","created":{"date-parts":[[2014,9,18]],"date-time":"2014-09-18T03:37:50Z","timestamp":1411011470000},"page":"543-552","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":6,"title":["Polycystic Kidneys and GM\n                    <sub>2<\/sub>\n                    Gangliosidosis-Like Disease in Neonatal Springboks (\n                    <i>Antidorcas marsupialis<\/i>\n                    )"],"prefix":"10.1177","volume":"52","author":[{"given":"V.","family":"Herder","sequence":"first","affiliation":[{"name":"Department of Pathology, University of Veterinary Medicine Hannover, Hannover, Germany"},{"name":"Center for Systems Neuroscience, Hannover, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Kummrow","sequence":"additional","affiliation":[{"name":"Erlebnis-Zoo Hannover, Hannover, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Leeb","sequence":"additional","affiliation":[{"name":"Vetsuisse Faculty, Institute of Genetics, University of Bern, Bern, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. 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