Enzyme kinetics and inhibition parameters of human leukocyte glucosylceramidase [PDF]
Heliyon, 2020 Glucosylceramidase (GCase) is a lysosomal enzyme that catalyzes the cleavage of β-glucosidic linkage of glucocerebroside (GC) into glucose and ceramide; thereby, plays an essential function in the degradation of complex lipids and the turnover of ...
Mesut Karatas +2 more
exaly +6 more sources
Identification of the Non-lysosomal Glucosylceramidase as β-Glucosidase 2 [PDF]
Journal of Biological Chemistry, 2007 The primary catabolic pathway for glucosylceramide is catalyzed by the lysosomal enzyme glucocerebrosidase that is defective in Gaucher disease patients. A distinct non-lysosomal glucosylceramidase has been described but its identity remained enigmatic for years.
Rolf G Boot +2 more
exaly +7 more sources
Function of saposin C in the reconstitution of glucosylceramidase by phosphatidylserine liposomes
FEBS Letters, 1993 The function of saposin C (Sap C), a glucosylceramidase activator protein, in the enzyme stimulation by phosphatidylserine (PS) liposomes has been investigated. Using gel filtration experiments evidence was obtained for Sap C binding to PS large unilamellar vesicles (LUV) but not to glucosylceramidase.
Anna Maria Vaccaro +2 more
exaly +5 more sources
Generation of Specific Deoxynojirimycin-type Inhibitors of the Non-lysosomal Glucosylceramidase [PDF]
Journal of Biological Chemistry, 1998 The existence of a non-lysosomal glucosylceramidase in human cells has been documented (van Weely, S., Brandsma, M., Strijland, A., Tager, J. M., and Aerts, J. M. F. G. (1993) Biochim. Biophys. Acta 1181, 55-62). Hypothetically, the activity of this enzyme, which is localized near the cell surface, may influence ceramide-mediated signaling processes ...
Herman S Overkleeft +2 more
exaly +8 more sources
The binding of glucosylceramidase to glucosylceramide is promoted by its activator protein
FEBS Letters, 1987 A protein activator of glucosylceramidase (EC 3.2.1.45) has been previously identified by us in human placenta [(1985) Biochim. Biophys. Acta 836, 157–166]. In the present paper we report that its function in vitro is to stimulate the binding of the enzyme to its substrate, glucosylceramide. After the purification step which frees the enzyme of most of
Anna Maria Vaccaro, Massimo Tatti
exaly +4 more sources
Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids [PDF]
FEBS Letters, 2000 The reconstitution of the activity of the lysosomal enzyme glucosylceramidase requires anionic phospholipids and, at least, a protein factor, saposin C (Sap C). We have previously proposed a mechanism for the glucosylceramidase activation [Vaccaro et al. (1993) FEBS Lett.
Rosa Salvioli +2 more
exaly +4 more sources
Autophagic dysregulation triggers innate immune activation in glucocerebrosidase deficiency [PDF]
Autophagy ReportsMutations in the GBA1 (glucosylceramidase beta 1) gene cause the most common lysosomal storage disorder, Gaucher disease (GD), characterized by the lysosomal accumulation of glucosylceramide and lysosomal dysfunction.
Magda L. Atilano +2 more
doaj +3 more sources
Effect of Expression of Human Glucosylceramidase 2 Isoforms on Lipid Profiles in COS-7 Cells [PDF]
Metabolites, 2020 Glucosylceramide (GlcCer) is a major membrane lipid and the precursor of gangliosides. GlcCer is mainly degraded by two enzymes, lysosomal acid β-glucosidase (GBA) and nonlysosomal β-glucosidase (GBA2), which may have different isoforms because of ...
Peeranat Jatooratthawichot +6 more
doaj +4 more sources
Synuclein Disorder-Related Genetic Determinants of Mild Behavioural Impairment in a Pre-Clinical Community Cohort. [PDF]
Int J Geriatr PsychiatryABSTRACT Background The GBA variant confers increased risk of synuclein disorders but it is unclear what impact it has in pre‐clinical groups. This study aimed to identify early psychiatric and cognitive manifestations amongst pre‐clinical GBA carriers in a community cohort.
Sander-Long M +5 more
europepmc +2 more sources
The role of lipid metabolism in neuronal senescence. [PDF]
FEBS Open BioDisrupted lipid metabolism, through alterations in lipid species or lipid droplet accumulation, can drive neuronal senescence. However, lipid dyshomeostasis can also occur alongside neuronal senescence, further amplifying tissue damage. Delineating how lipid‐induced senescence emerges in neurons and glial cells, and how it contributes to ageing and ...
Tsagkari D +2 more
europepmc +2 more sources