{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T02:57:57Z","timestamp":1761101877494},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2009,3,4]],"date-time":"2009-03-04T00:00:00Z","timestamp":1236124800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2009,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>In tissue lesions of type I Gaucher patients, characteristic lipid\u2010laden macrophages, \u2018Gaucher cells\u2019, are surrounded by inflammatory phagocytes. Gaucher cells secrete the elevated plasma chitotriosidase. The elevated plasma MIP\u20101\u03b2 in Gaucher patients stems from the phagocytes surrounding the Gaucher cells. Plasma chitotriosidase and MIP\u20101\u03b2 decrease upon successful enzyme replacement therapy (ERT) with mannose\u2010terminated recombinant glucocerebrosidase (alglucerase). Previous histochemical analysis of Gaucher spleens revealed that Gaucher cells express little mannose receptor, in contrast to surrounding phagocytes. We therefore investigated the corrective effects of ERT on plasma MIP\u20101\u03b2 and chitotriosidase in more detail. We also compared effects of one year of treatment with a relatively low dose and a relatively high dose of ERT. A more rapid correction in plasma MIP\u20101\u03b2, compared to chitotriosidase, was observed in most patients on low\u2010dose ERT. Correction of plasma MIP\u20101\u03b2 and chitotriosidase levels was more pronounced in the higher\u2010dosed patient group. Upon prolonged treatment, differences in the effects of enzyme dose were no longer significant. Normalization of plasma MIP\u20101\u03b2 and chitotriosidase levels was attained in the majority of patients. In conclusion, ERT with mannose\u2010terminated gluocerebrosidase results in prominent corrections of plasma chitotriosidase, a marker of Gaucher cells, and in particular of plasma MIP\u20101\u03b2, a marker of inflammatory phagocytes. The sharper response in plasma MIP\u20101\u03b2 to ERT is in line with the observation that especially phagocytes surrounding Gaucher cells express mannose\u2010receptors.<\/jats:p>","DOI":"10.1007\/s10545-009-1064-5","type":"journal-article","created":{"date-parts":[[2009,3,2]],"date-time":"2009-03-02T19:35:41Z","timestamp":1236022541000},"page":"274-279","source":"Crossref","is-referenced-by-count":7,"title":["Different dose\u2010dependent correction of MIP\u20101\u03b2 and chitotriosidase during initial enzyme replacement therapy"],"prefix":"10.1002","volume":"32","author":[{"given":"M. J.","family":"van Breemen","sequence":"first","affiliation":[{"name":"Department of Medical Biochemistry, K1\u2013262 University of Amsterdam, Academic Medical Center Amsterdam 1105 AZ The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"de Fost","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism Academic Medical Center Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Maas","sequence":"additional","affiliation":[{"name":"Department of Radiology Academic Medical Center Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. G.","family":"Wiersma","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism Academic Medical Center Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. E. M.","family":"Hollak","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism Academic Medical Center Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L. W.","family":"Poll","sequence":"additional","affiliation":[{"name":"Institute of Radiology Heinrich\u2010Heine University Duisburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"vom Dahl","sequence":"additional","affiliation":[{"name":"Clinic for Internal Medicine, University Hospital Heinrich\u2010Heine University Cologne Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. G.","family":"Boot","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry, K1\u2013262 University of Amsterdam, Academic Medical Center Amsterdam 1105 AZ The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. M. F. 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