Glucosylsphingosine is a cytotoxic compound. Accumulation of glucosylsphingosine in brain and other tissues occurs in patients with Gaucher disease, which is an inherited deficiency of lysosomal glucocerebrosidase, which converts glucosylsphingosine to glucose and sphingosine.
Gaucher disease (GD) is caused by mutations on the GBA1 gene leading to deficiency in acid β-glucosidase (GCase) and subsequent accumulation of its substrates, glucosylceramide (GlcC) and glucosylsphingosine (GlcS). GlcS in plasma has been proposed as a highly sensitive and
Gaucher disease (GD) is caused by a deficiency of β-glucosidase (GCase), leading to accumulation of glucosylceramide (GlcC) and glucosylsphingosine (Lyso-Gb1). Lyso-Gb1 is a reliable biomarker for GD. This study aims to develop a simple, effective and accurate method for the
A new series of 9-plex chemical isotope-labeling reagents, levofloxacin-based mass tags (LMTs) named as LMT359, 360, 361, 362, 363, 373, 375, 376, and 378, was firstly designed and synthesized for the high-throughput labeling of globotriaosylsphingosine (lyso-Gb3), a disease biomarker of
Splenectomy in children with the Norrbottnian type of Gaucher disease is followed by increased blood levels of glucosylceramide and impaired neurological and mental status. High blood levels are associated with an increased accumulation of glucosylceramide in perivascular Gaucher cells in
Gaucher disease is a glycolipid storage disorder characterized by accumulation of glucocerebroside in the liver, spleen, and bones, and caused by a deficiency of glucocerebrosidase. Glucocerebrosidase cDNA has been cloned and sequenced, and much has been learned about the synthesis
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