caa a22 4500 467933448 CHVBK 20180406152950.0 cr unu---uuuuu 170328e20060801xx s 000 0 eng 10.1007/s10545-006-0318-8 doi (NATIONALLICENCE)springer-10.1007/s10545-006-0318-8 CCL18: A urinary marker of Gaucher cell burden in Gaucher patients [Elektronische Daten] [Rolf Boot, Marri Verhoek, Mirjam Langeveld, G. Renkema, Carla Hollak, Jan Weening, Wilma Donker-Koopman, Johanna Groener, Johannes Aerts] Glucosylceramide-laden tissue macrophages in Gaucher patients secrete large quantities of chitotriosidase and CC chemokine ligand 18 (CCL18), resulting in markedly increased plasma levels. We have comparatively investigated the occurrence of both parameters in plasma and urine samples of Gaucher patients. Chitotriosidase was high in urine samples of some symptomatic patients, but elevations did not correlate with increased plasma concentrations. Urinary chitotriosidase was particularly high in a patient with severe kidney involvement and local storage cell infiltration. Urinary levels of CCL18 were also highly elevated in samples from Gaucher patients as compared to controls. The median value of the CCL18/creatinine ratio in urine samples of 31 Gaucher patients was 143.3 pg/μmol (range 32-551) and in those of 12 normal subjects was 4.1 pg/μmol (range 1.3-6.8). In sharp contrast to chitotriosidase, increases in the low-molecular-mass chemokine CCL18 in urine and plasma specimens of Gaucher patients correlated well. A correlation was also observed for reductions in urinary and plasma CCL18 following therapy. It is concluded that assessment of urinary CCL18 of Gaucher patients gives insight into the total body burden on Gaucher cells, whereas that of chitotriosidase does not. Urinary chitotriosidase appears rather to be a reflection of renal pathology. SSIEM and Springer, 2006 Boot Rolf Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Verhoek Marri Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Langeveld Mirjam Department of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Renkema G. Institute of Medical Technology, University of Tampere, Tampere, Finland aut Hollak Carla Department of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Weening Jan Department of Pathology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Donker-Koopman Wilma Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Groener Johanna Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Aerts Johannes Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/4(2006-08-01), 564-571 0141-8955 29:4<564 2006 29 10545 https://doi.org/10.1007/s10545-006-0318-8 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0318-8 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Boot Rolf Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Verhoek Marri Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Langeveld Mirjam Department of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Renkema G. Institute of Medical Technology, University of Tampere, Tampere, Finland aut NATIONALLICENCE 700 1- Hollak Carla Department of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Weening Jan Department of Pathology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Donker-Koopman Wilma Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Groener Johanna Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Aerts Johannes Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/4(2006-08-01), 564-571 0141-8955 29:4<564 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer