SEARCH:   Advanced

Prepublished online as a Blood First Edition Paper on September 11, 2003; DOI 10.1182/blood-2003-05-1612. This Article Full Text (PDF) All Versions of this Article: 2003-05-1612v1 103/1/33    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Boot, R. G Articles by Aerts, J. M F G Search for Related Content PubMed PubMed Citation Articles by Boot, R. G Articles by Aerts, J. M F G Social Bookmarking                   What's this? Submitted May 20, 2003 Accepted July 15, 2003 Marked elevation of the chemokine CCL18 / PARC in Gaucher disease: a novel surrogate marker for assessing therapeutic intervention Rolf G Boot*, Marri Verhoek, Maaike de Fost, Carla E M Hollak, Mario Maas, Boris Bleijlevens, Marielle J van Breemen, Marjan van Meurs, Leonie A Boven, Jon D Laman, Mary Teresa Moran, Timothy M Cox, and Johannes M F G Aerts Department of Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands Department of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands Department of Radiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands Department of Immunology, Erasmus Medical Center, University of Rotterdam, Rotterdam, The Netherlands Department of Medicine, Addenbrooke's Hospital, University of Cambridge, Cambridge, United Kingdom * Corresponding author; email: r.g.boot{at}amc.uva.nl. Gaucher disease is characterized by storage of glucosylceramide in lysosomes of tissue macrophages as the result of an autosomal recessively inherited deficiency in glucocerebrosidase. Progressive accumulation of these glycolipid-laden Gaucher cells causes a variety of debilitating symptoms. The disease can be effectively treated by costly intravenous infusions with recombinant glucocerebrosidase. Chitotriosidase is massively secreted by Gaucher cells and its plasma levels are employed to monitor efficacy of enzyme therapy. Broad scale application is hampered by the common genetic defect in this surrogate marker. We report that in plasma of symptomatic Gaucher patients the chemokine CCL18 is on average 29 fold elevated, without overlap between patients' and control values (median control plasma level is 33 ng/ml; range: 10-72, median Gaucher plasma level is 948 ng/ml; range: 237-2285). Plasma CCL18 concentrations decrease during therapy, comparably to chitotriosidase levels. Immunohistochemistry demonstrates that Gaucher cells are the prominent source of CCL18. Plasma CCL18 levels can serve as alternative surrogate marker for storage cells in Gaucher patients and monitoring of plasma CCL18 levels proves to be very useful in determination of therapeutic efficacy, especially in patients that are deficient in chitotriosidase activity. The potential physiological consequences of chronically elevated CCL18 in Gaucher patients are discussed. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Prasse, C. Probst, E. Bargagli, G. Zissel, G. B. Toews, K. R. Flaherty, M. Olschewski, P. Rottoli, and J. Muller-Quernheim Serum CC-Chemokine Ligand 18 Concentration Predicts Outcome in Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., April 15, 2009; 179(8): 717 - 723. [Abstract] [Full Text] [PDF] M. Di Rocco, F. Giona, F. Carubbi, S. Linari, F. Minichilli, R. O. Brady, G. Mariani, and M. D. Cappellini A new severity score index for phenotypic classification and evaluation of responses to treatment in type I Gaucher disease Haematologica, August 1, 2008; 93(8): 1211 - 1218. [Abstract] [Full Text] [PDF] A. W T van Lieshout, J. Fransen, M. Flendrie, A. M M Eijsbouts, F. H J van den Hoogen, P. L C M van Riel, and T. R D J Radstake Circulating levels of the chemokine CCL18 but not CXCL16 are elevated and correlate with disease activity in rheumatoid arthritis Ann Rheum Dis, October 1, 2007; 66(10): 1334 - 1338. [Abstract] [Full Text] [PDF] J. P. C. Vissers, J. I. Langridge, and J. M. F. G. Aerts Analysis and Quantification of Diagnostic Serum Markers and Protein Signatures for Gaucher Disease Mol. Cell. Proteomics, May 1, 2007; 6(5): 755 - 766. [Abstract] [Full Text] [PDF] J. Schmitz, L. W. Poll, and S. v. Dahl Therapy of adult Gaucher disease Haematologica, February 1, 2007; 92(2): 148 - 152. [Full Text] [PDF] C. S. Tan, A. Ploner, A. Quandt, J. Lehtio, and Y. Pawitan Finding regions of significance in SELDI measurements for identifying protein biomarkers Bioinformatics, June 15, 2006; 22(12): 1515 - 1523. [Abstract] [Full Text] [PDF] E. Schutyser, A. Richmond, and J. Van Damme Involvement of CC chemokine ligand 18 (CCL18) in normal and pathological processes J. Leukoc. Biol., July 1, 2005; 78(1): 14 - 26. [Abstract] [Full Text] [PDF] R. van der Voort, M. Kramer, E. Lindhout, R. Torensma, D. Eleveld, A. W. T. van Lieshout, M. Looman, T. Ruers, T. R. D. J. Radstake, C. G. Figdor, et al. Novel monoclonal antibodies detect elevated levels of the chemokine CCL18/DC-CK1 in serum and body fluids in pathological conditions J. Leukoc. Biol., May 1, 2005; 77(5): 739 - 747. [Abstract] [Full Text] [PDF]

	Copyright © 2003 by American Society of Hematology         Online ISSN: 1528-0020