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Zus\u00e4tzlich bestanden eine hochgradige Degeneration und ein Verlust von Purkinjeund inneren K\u00f6rnerzellen des Kleinhirns. Das akkumulierte PAS-positive, argyrophile, autofluoreszierende Material wies elektronenmikroskopisch eine lamell\u00e4re Struktur auf und wurde als Lipofuszin interpretiert. Eine genetische Analyse best\u00e4tigte das Vorliegen einer Variante im ARSG-Gen, das f\u00fcr das lysosomale Enzym Arylsulfatase G kodiert. Der Fallbericht beschreibt eine neuronale Zeroidlipofuszinose bei einem adulten Hund, die \u00c4hnlichkeiten mit der Kufs-Krankheit des Menschen aufweist.<\/jats:p>","DOI":"10.15654\/tpk-150766","type":"journal-article","created":{"date-parts":[[2016,10,25]],"date-time":"2016-10-25T08:13:42Z","timestamp":1477383222000},"page":"431-437","source":"Crossref","is-referenced-by-count":3,"title":["Neuronale Zeroidlipofuszinose bei einem adulten American Staffordshire Terrier"],"prefix":"10.15654","volume":"44","author":[{"given":"Anna","family":"Nolte","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aimara","family":"Bello","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michaela","family":"Dr\u00f6gem\u00fcller","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tosso","family":"Leeb","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva","family":"Brockhaus","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Baumg\u00e4rtner","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Wohlsein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2017,12,20]]},"reference":[{"key":"10.15654\/TPK-150766-1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0914206107"},{"key":"10.15654\/TPK-150766-2","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9975(82)90022-6"},{"key":"10.15654\/TPK-150766-3","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2006.02.016"},{"key":"10.15654\/TPK-150766-4","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2005.11.005"},{"key":"10.15654\/TPK-150766-5","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9075-9"},{"key":"10.15654\/TPK-150766-6","doi-asserted-by":"publisher","DOI":"10.1111\/avj.12211"},{"key":"10.15654\/TPK-150766-7","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.genom.6.080604.162334"},{"key":"10.15654\/TPK-150766-8","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.23710"},{"key":"10.15654\/TPK-150766-9","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2011.02.009"},{"key":"10.15654\/TPK-150766-10","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M709917200"},{"key":"10.15654\/TPK-150766-11","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.04.001"},{"key":"10.15654\/TPK-150766-12","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320310617"},{"key":"10.15654\/TPK-150766-13","doi-asserted-by":"publisher","DOI":"10.1186\/s12917-014-0181-z"},{"key":"10.15654\/TPK-150766-14","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2006.06.010"},{"key":"10.15654\/TPK-150766-15","doi-asserted-by":"publisher","DOI":"10.1024\/0036-7281.145.8.369"},{"key":"10.15654\/TPK-150766-16","doi-asserted-by":"publisher","DOI":"10.1111\/j.1939-1676.2008.0138.x"},{"key":"10.15654\/TPK-150766-17","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2008.11.004"},{"key":"10.15654\/TPK-150766-18","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-0813.1997.tb13839.x"},{"key":"10.15654\/TPK-150766-19","doi-asserted-by":"publisher","DOI":"10.1024\/0036-7281\/a000623"},{"key":"10.15654\/TPK-150766-20","doi-asserted-by":"publisher","DOI":"10.1155\/2011\/198042"},{"key":"10.15654\/TPK-150766-21","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2004.12.038"},{"key":"10.15654\/TPK-150766-22","doi-asserted-by":"publisher","DOI":"10.2460\/ajvr.2005.66.67"},{"key":"10.15654\/TPK-150766-23","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0683-y"},{"key":"10.15654\/TPK-150766-24","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0133916"},{"key":"10.15654\/TPK-150766-25","doi-asserted-by":"publisher","DOI":"10.1292\/jvms.66.1453"},{"key":"10.15654\/TPK-150766-26","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320310616"},{"key":"10.15654\/TPK-150766-27","unstructured":"Lorenz MD, Coates J, Kent M. 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