{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T02:00:42Z","timestamp":1791165642657,"version":"4.1.0"},"reference-count":0,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2017,8,11]],"date-time":"2017-08-11T00:00:00Z","timestamp":1502409600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2017,8,11]],"date-time":"2017-08-11T00:00:00Z","timestamp":1502409600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Enzyme"],"abstract":"<jats:p>Canine mucopolysaccharidosis type VII results from deficient activity of lysosomal\n\u00df-glucuronidase. Residual enzymatic activity (0.2-1.7% of normal) was detected in\ntissue homogenates from affected dogs. In contrast, serum and urine from affected animals\nhad up to 15% residual activity. To further characterize the nature of the defective enzyme,\nhepatic \u00df-glucuronidase was partially purified from normal and MPS VII dogs for determination\nof their physical and kinetic properties. About 65% of the total \u00df-glucuronidase in\nnormal canine liver required detergent for solubilization (i.e., membrane-associated),\nwhereas only 22% of the residual activity in canine MPS VII liver was membrane-associated.\nCompared to the normal hepatic enzyme, the K(m) towards 4-methylumbelliferyl-\u00df-glucuronide\nwas markedly increased in MPS VII dogs (i.e., 0.48 versus &gt; 2.5 mmol\/l). In contrast,\nthe thermo-, cryo-, and pH stability properties, as well as the pH optimum (\u2248 4.6), were\nessentially unaffected. In addition, the canine MPS VII hepatic residual activity was unresponsive\nto sulfhydryl reducing reagents and divalent cations, despite the fact that incubation\nof normal canine \u00df-glucuronidase with dithiothreitol and magnesium and\/or calcium\nenhanced the activity more than 15-fold.<\/jats:p>","DOI":"10.1159\/000469027","type":"journal-article","created":{"date-parts":[[2017,8,11]],"date-time":"2017-08-11T17:21:57Z","timestamp":1502472117000},"page":"174-180","source":"Crossref","is-referenced-by-count":18,"title":["Characterization of the Defective \u00df-Glucuronidase Activity in\nCanine Mucopolysaccharidosis Type VII"],"prefix":"10.1159","author":[{"given":"Edward","family":"Schuchman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamitza","family":"Toroyan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Haskins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Desnick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2017,8,11]]},"container-title":["Enzyme"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000469027","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T12:46:17Z","timestamp":1777553177000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000469027"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,8,11]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1159\/000469027","archive":["Portico"],"relation":{},"ISSN":["0013-9432","2504-2564"],"issn-type":[{"value":"0013-9432","type":"print"},{"value":"2504-2564","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,8,11]]}}}