... glucosyl ceramide beta 1-4 galactosyltransferase, UDP-Gal:glucosylceramide beta1->4galactosyltransferase, GalT-2, UDP-galactose ... Glucosylceramide+beta-1,4-galactosyltransferase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal ... Trinchera M, Fiorilli A, Ghidoni R (March 1991). "Localization in the Golgi apparatus of rat liver UDP-Gal:glucosylceramide ... Glucosylceramide beta-1,4-galactosyltransferase (EC 2.4.1.274, lactosylceramide synthase, uridine diphosphate-galactose: ...
Glucosylceramide Synthase Inhibitors. Class Summary. These agents inhibit the enzyme glucosylceramide synthase, the initial ... Eliglustat is a specific inhibitor of glucosylceramide synthase, thereby reducing production of glucosylceramide. It is ... The glucosylceramide synthase inhibitors, miglustat and eliglustat, were approved for adults with type 1 Gaucher disease in ... Reduces GSL production by inhibiting glucosylceramide synthase. Reduces spleen and liver volume and increases hemoglobin and ...
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Plasma ceramides and glucosylceramides predict cognitive decline among cognitively normal Parkinsons disease patients. M.M. ... Plasma ceramides and glucosylceramides were determined by LC/ESI/MS/MS. Linear mixed models were used to assess the association ... For example, each log unit increase in the ceramide to glucosylceramide C18:0 ratio was associated with greater decline in all ... Conclusions: Our results suggest that a higher plasma ceramide to glucosylceramide C18:0 ratio is predictive of greater rates ...
We treated both multidrug-resistant cell lines with the glucosylceramide synthase inhibitors PDMP (d-threo-1-phenyl-2- ... However, the N-alkylated iminosugars C9DGJ and C4DGJ, which are more selective glucosylceramide synthase inhibitors than PDMP, ... Our results suggest that (a) inhibition of glucosylceramide synthase does not reverse multidrug resistance and (b) the ... Inhibiting glycolipid biosynthesis by blocking glucosylceramide synthase has been proposed to reverse drug resistance in MDR ...
Glucosylceramide beta-glucosidase deficiency. *Glucosylceramide lipidosis. *Kerasin histiocytosis. *Kerasin lipoidosis. * ...
Adipocytes harbor a glucosylceramide biosynthesis pathway involved in iNKT cell activation Rakhshandehroo M., van Eijkeren RJ ...
Ahn, Y. M., Lee, W. W., Jung, J. H., Lee, S. G., & Honga, J. (2009). Structural determination of glucosylceramides isolated ... Ahn YM, Lee WW, Jung JH, Lee SG, Honga J. Structural determination of glucosylceramides isolated from marine sponge by fast ... Five glucosylceramides (GlcCers) were isolated by reversed phase high-performance liquid chromatography from the MeOH extracts ... Ahn, YM, Lee, WW, Jung, JH, Lee, SG & Honga, J 2009, Structural determination of glucosylceramides isolated from marine sponge ...
Glucosylceramide Beta-Glucosidase Deficiency Disease; Neuronopathic Gaucher Disease; Gaucher Disease Type 1; Gaucher Disease ... gaucher disease; Cerebroside Lipidosis Syndrome; Glucocerebrosidase Deficiency Disease; Glucosylceramide Beta-Glucosidase ...
Plasma Ceramide and Glucosylceramide Metabolism Is Altered in Sporadic Parkinsons Disease and Associated with Cognitive ... Plasma Ceramide and Glucosylceramide Metabolism Is Altered in Sporadic Parkinsons Disease and Associated with Cognitive ... Plasma Ceramide and Glucosylceramide Metabolism Is Altered in Sporadic Parkinsons Disease and Associated with Cognitive ... Plasma Ceramide and Glucosylceramide Metabolism Is Altered in Sporadic Parkinsons Disease and Associated with Cognitive ...
Oral intake of glucosylceramide improves relatively higher level of transepidermal water loss in mice and healthy human ... Structural determination of glucosylceramides in the distillation remnants of shochu, the Japanese traditional liquor, and its ... Quantitation and structural determination of glucosylceramides contained in sake lees. J. Oleo Sci. 2014, 63, 15-23. [Google ... Since the glycosylceramide contained in koji contains the same species of glucosylceramide contained in konjac [23], the ...
Dopamine Oxidation, Glucosylceramide and α-Synuclein 8:05 pm - 8:15 pm. Discussion ...
Targeting Glucosylceramide Synthase Synergizes With C 6 -Ceramide Nanoliposomes to Induce Apoptosis in Natural Killer Cell ... The complex glycosphingolipids are hydrolyzed to glucosylceramide and galactosylceramide. These lipids are then hydrolyzed by ... to form ceramide-1-phosphate or it can be glycosylated by glucosylceramide synthase to form glycosphingolipids (cerebrosides, ...
Mutated ATP10B increases Parkinsons disease risk by compromising lysosomal glucosylceramide export Shaun Martin, Stefanie ...
... epidermal keratinocytes from patients that carry ABCA12 mutations have demonstrated disturbed intracytoplasmic glucosylceramide ...
Mutated ATP10B increases Parkinsons disease risk by compromising lysosomal glucosylceramide export Shaun Martin, Stefanie ...
The disease occurs when the lipid glucosylceramide accumulates in the bone marrow, lungs, spleen, liver, and sometimes the ... In contrast, substrate reduction therapy works by inhibiting glucosylceramide synthase, slowing the production of ... glucosylceramide. The FDA has approved another agent for substrate reduction, miglustat (Zavesca, Actelion), but only for ... that can be given intravenously to supplement the patients glucocerebrosidase and break down accumulated glucosylceramide. ...
Glucosylceramide synthase deficiency in the heart compromises β1-adrenergic receptor trafficking. Linda Andersson, Mathieu ...
... glucosylceramide, and resistance to cancer treatment [229,230,231]. Levels of glucosylceramide are regulated by the activity of ... Lucci, A.; Cho, W.I.; Han, T.Y.; Giuliano, A.E.; Morton, D.L.; Cabot, M.C. Glucosylceramide: A marker for multiple-drug ... Liu, Y.Y.; Han, T.Y.; Giuliano, A.E.; Cabot, M.C. Expression of glucosylceramide synthase, converting ceramide to ... glucosylceramide synthase) in breast cancer cells. J. Surg. Oncol. 2011, 104, 466-471. [Google Scholar] [CrossRef] ...
Glucosylceramide and glucosylsphingosine quantitation by liquid chromatography-tandem mass spectrometry to enable in vivo ... glucosylceramide (GlcCer), galactosylceramide (GalCer), sulfatides, and sphingomyelins (SMs). There may also be a decrease of ... Levels of glucosylceramide (GlcCer), galactosylceramide (GalCer), glucosylsphingosine (GlcSph), and galactosylsphingosine ( ...
Glucosylceramide Synthase ; Hib: Haemophilus influenzae type b; NSCLC: non small cell lung cancer; MM: multiple myeloma; COPD: ...
Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease Authors: MK Pandey, TA Burrow, R ...
Glucosylceramide modifies the LPS-induced inflammatory response in macrophages and the orientation of the LPS/TLR4 complex in ...
PDMP This product is a glucosylceramide synthase inhibitor, an enzyme that is essential for the synthesis of a very large ... This product is a glucosylceramide synthase inhibitor, an enzyme that is essential for the synthesis of a very large number of ... "Effects of D-threo-PDMP, an inhibitor of glucosylceramide synthetase, on expression of cell surface glycolipid antigen and ... 3. N. Radin, et al., "Structural and stereochemical studies of potent inhibitors of glucosylceramide synthase and tumor cell ...
The resulting deficit in GCase activity leads to accumulation of the cellular fats glucosylceramide and glucosylsphingosine. ... The resulting deficit in GCase activity leads to accumulation of the cellular fats glucosylceramide and glucosylsphingosine. ...
Miglustat is a competitive and reversible inhibitor of glucosylceramide synthase, the initial enzyme in a series of reactions ... glucosylceramide) that builds up due to this condition. This can help improve symptoms of Gauchers disease, such as tiredness ... resulting in synthesis of most glygosphingolipids; decreases rate of glycosphingolipid glucosylceramide formation ... the enzyme that mediates glycosphingolipid glucosylceramide degradation ...
Glucosylceramide Beta-Glucosidase Deficiency Disease. *Acid beta-Glucosidase Deficiency. *Acid beta-Glucosidase Deficiency ...
There was also a 1.7-fold increase in BBM glucosylceramide content (528 +/- 63 vs. 312 +/- 41 ng/mg BBM protein in control, P ... These cultures upregulate glucosylceramide synthase and make normal-appearing lipid lamellar bilayers. The epidermal ... 0.02). To determine whether the alteration in glucosylceramide content per se played a functional role in the decrease in Na-Pi ... cotransport, control rats were treated with the glucosylceramide synthase inhibitor, D-threo-1-phenyl-2-decanoyl-amino-3- ...
Ceramides & Glucosylceramides. 4 mg. *. Amino Acid Complex. 120 mg. *. *Daily Value Not Established. ***Percent Daily Values ...