2008
DOI: 10.1177/039463200802100404
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CD203c is Overexpressed on Neoplastic Mast Cells in Systemic Mastocytosis and is Upregulated upon IgE Receptor Cross-Linking

Abstract: The ectoenzyme E-NPP3 (CD203c) has recently been identified as a novel activation-linked cell surface antigen on basophils. In the present study, we examined expression of CD203c on normal mast cells (MC) and bone marrow (bm) MC derived from 85 patients with systemic mastocytosis (SM), including cases with indolent SM (ISM, n=72), SM with associated clonal hematologic non-MC-lineage disease (SM-AHNMD, n=6), aggressive SM (ASM, n=3), and mast cell leukemia (MCL, n=4). Surface expression of CD203c was analyzed b… Show more

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Cited by 46 publications
(48 citation statements)
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“…This protein defines the basophil lineage in the bone marrow and shows an elevated distribution on the cell surface of activated basophils. It can serve as a marker for the estimation of hypersensitivity to allergens [339][340][341][342]. Recent experiments implicate a dual role of NPP3 in vascular smooth muscle calcification by producing (together with NPP1) PP i from ATP on the one hand and subsequently hydrolyzing (together with TNAP) the PP i produced [7].…”
Section: General Properties and Functional Rolementioning
confidence: 99%
“…This protein defines the basophil lineage in the bone marrow and shows an elevated distribution on the cell surface of activated basophils. It can serve as a marker for the estimation of hypersensitivity to allergens [339][340][341][342]. Recent experiments implicate a dual role of NPP3 in vascular smooth muscle calcification by producing (together with NPP1) PP i from ATP on the one hand and subsequently hydrolyzing (together with TNAP) the PP i produced [7].…”
Section: General Properties and Functional Rolementioning
confidence: 99%
“…Mouse bone marrow-derived mast cells (BMMCs) highly express CD39 and CD73 (Bulanova E., unpublished observation) and therefore might contribute to the concentration of ATP and its derivatives, ADP and AMP, in the microenvironment. Importantly, E-NPP3 (CD203c) was recently identified as a novel activation-linked surface marker on human mast cells and basophils [32]. It is upregulated in response to IgE crosslinking and highly expressed on neoplastic mast cells in patients with systemic mastocytosis [32].…”
Section: Mast Cellsmentioning
confidence: 99%
“…Importantly, E-NPP3 (CD203c) was recently identified as a novel activation-linked surface marker on human mast cells and basophils [32]. It is upregulated in response to IgE crosslinking and highly expressed on neoplastic mast cells in patients with systemic mastocytosis [32]. The expression and role of these and other ATP-hydrolyzing enzymes for the biology of mast cells remains to be elucidated.…”
Section: Mast Cellsmentioning
confidence: 99%
“…Опухолевые ТК, как правило, характеризуются аномальной экспрессией CD25, с или без CD2 [10,[32][33][34][35][36][37], CD123, CD203c, CD63, CD69 или CD45, а также низким уровнем экспрессии CyB12 [34,38]. ТК при СМЦ характеризуются аберрантной анти-генной экспрессией, что позволяет проводить четкую дифферен-циальную диагностику от нормальных ТК с чувствительностью выше 98% и специфичностью 100% [32].…”
Section: таблица в-и с-признаки мц (воз 2008)unclassified