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Soluble mannose receptor: A potential biomarker in Gaucher disease

Beaton, B; Hughes, DA; (2024) Soluble mannose receptor: A potential biomarker in Gaucher disease. European Journal of Haematology 10.1111/ejh.14171. (In press). Green open access

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Abstract

Purpose: Soluble mannose receptor (sMR) relates to mannose receptor expression on macrophages, and is elevated in inflammatory disorders. Gaucher disease (GD) has altered macrophage function and utilises mannose receptors for enzyme replacement therapy (ERT) endocytosis. sMR has not previously been studied in GD. Methods: sMR was measured by ELISA and correlated with GD clinical features including spleen and liver volume, haemoglobin and platelet count, bone marrow burden (BMB) scores and immunoglobulin levels. sMR was compared with biomarkers of GD: chitotriosidase, lyso-GL1, PARC, CCL3, CCL4, osteoactivin, serum ACE and ferritin. Results: Median sMR in untreated GD patients was 303.0 ng/mL compared to post-treatment 190.9 ng/mL (p =.02) and healthy controls 202 ng/mL. Median sMR correlated with median spleen volume 455 mL (r =.70, p =.04), liver volume 2025 mL (r =.64, p =.04), BMB 7 (r =.8, p =.03), IgA 1.9 g/L (r =.54, p =.036), IgG 9.2 g/L (r =.57, p =.027), IgM 1.45 g/L (r =.86, p <.0001), with inverse correlation to median platelet count of 125 × 109/L (r = −.47, p =.08) and haemoglobin of 137 g/L (r = −.77, p =.0008). sMR correlated with established biomarkers: osteoactivin 107.8 ng/mL (r =.58, p =.0006), chitotriosidase 3042 nmol/mL/h (r =.52, p =.0006), PARC 800 ng/mL (r =.67, p =.0068), ferritin 547 μg/L (r =.72, p =.002) and CCL3 50 pg/mL (r =.67, p =.007). Conclusions: sMR correlates with clinical features and biomarkers of GD and reduces following therapy.

Type: Article
Title: Soluble mannose receptor: A potential biomarker in Gaucher disease
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/ejh.14171
Publisher version: http://dx.doi.org/10.1111/ejh.14171
Language: English
Additional information: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. © 2024 The Authors. European Journal of Haematology published by John Wiley & Sons Ltd.
Keywords: Gaucher disease, biomarker, soluble mannose receptor
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Haematology
URI: https://discovery.ucl.ac.uk/id/eprint/10186451
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