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. 2020 Mar 9;9(3):736. doi: 10.3390/jcm9030736

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© 2020 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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(A) Structure of glucosylceramide (GlcCer) and degradation by GCase to glucose and ceramide. (B) Catalytic activity GCase: Hydrolyzation of β-glucosides and transglucosylation activity. (C) Occurrence of Gaucher cells and the biomarkers they secrete in plasma. (D) Metabolic adaptations to GCase deficiency: increase of GlcCer as a result of lack of degradation by GCase. Accumulated GlcCer is converted by ASAH1 to glucosylsphingosine, Glucosylated cholesterol (GlcChol) formed by GBA2 increases, and GM3 levels rise because increased anabolism by glycosyltransferases to complex GSLs. Enzymes are depicted in green. ASAH1: acid ceramidase, GBA2: cytosolic β-glucosidase, GCase: β-glucocerebrosidase, GCS: glucosylceramide synthase.