{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T21:01:46Z","timestamp":1761512506136},"reference-count":17,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2010,12,23]],"date-time":"2010-12-23T00:00:00Z","timestamp":1293062400000},"content-version":"vor","delay-in-days":1027,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biotech and App Biochem"],"published-print":{"date-parts":[[2008,3]]},"abstract":"<jats:p>MPS VI (mucopolysaccharidosis type VI) is a lysosomal storage disease in which deficient activity of the enzyme <jats:italic>N<\/jats:italic>\u2010acetylgalactosamine 4\u2010sulfatase [ASB (arylsulfatase B)] impairs the stepwise degradation of the GAG (glycosaminoglycan) dermatan sulfate. Clinical studies of ERT (enzyme replacement therapy) by using rhASB (recombinant human ASB) have been reported with promising results. The release of GAG into the urine is currently used as a biomarker of disease, reflecting in some cases disease severity and in all cases therapeutic responsiveness. Using RNA studies in four Italian patients undergoing ERT, we observed that TNF\u03b1 (tumour necrosis factor \u03b1) might be a biomarker for MPS VI responsive to therapy. In addition to its role as a potential biomarker, TNF\u03b1 expression could provide insights into the possible pathophysiological mechanisms underlying the mucopolysaccharidoses.<\/jats:p>","DOI":"10.1042\/ba20070093","type":"journal-article","created":{"date-parts":[[2008,2,18]],"date-time":"2008-02-18T11:26:19Z","timestamp":1203333979000},"page":"219-223","source":"Crossref","is-referenced-by-count":17,"title":["Molecular markers for the follow\u2010up of enzyme\u2010replacement therapy in mucopolysaccharidosis type VI disease"],"prefix":"10.1002","volume":"49","author":[{"given":"Paola Di","family":"Natale","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guglielmo R. 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