Results 131 to 140 of about 9,362 (180)

Sinbaglustat is efficacious in GM2 gangliosidosis primarily through inhibition of GBA2 rather than GCS. [PDF]

open access: yesMol Ther Adv
Steiner MA   +8 more
europepmc   +1 more source

The Role of Glycosphingolipids in Autoimmune Manifestations and Myeloma in Gaucher Disease

open access: yes
Pavlova E   +5 more
europepmc   +1 more source

Glucosylceramide synthase and apoptosis

Biochimica Et Biophysica Acta - Molecular and Cell Biology of Lipids, 2002
Glucosylceramide synthase (GCS) is an enzyme inherent to ceramide metabolism. The enzyme catalyzes the transfer of glucose to ceramide, the first committed step in glycolipid biosynthesis. Known for many years as a branch point enzyme directing synthesis of cerebrosides and gangliosides, GCS has recently been implicated in the cytotoxic response of ...
Myles C Cabot
exaly   +3 more sources

Glucosylceramide synthase and glycosphingolipid synthesis

Trends in Cell Biology, 1998
In mammalian cells, there are two major classes of sphingolipids---sphingomyelin and glycosphingolipids (GSLs)--both of which are synthesized from the hydrophobic molecule ceramide. The synthesis of most GSLs begins with glucosylation of ceramide to form glucosylceramide (GlcCer), which, in turn, serves as the source of 300-400 GSLs.
Yoshio Hirabayashi, Shinichi Ichikawa
exaly   +3 more sources

Inhibitors of Glucosylceramide Synthase

2023
Glucosylceramide synthase can be targeted by high affinity small molecular weight inhibitors for the study of glycosphingolipid metabolism and function or for the treatment of glycosphingolipid storage disorders, including Gaucher and Fabry disease. This work is exemplified by the discovery and development of eliglustat tartrate, the first stand-alone ...
James A, Shayman   +2 more
openaire   +2 more sources

Immunogenic properties of glucosylceramide

Molecular Immunology, 1979
Abstract Specific antibodies against glucosylceramide were elicited in rabbits by repeated intravenous injection of a mixture of hapten-cholesterol-human albumin (0.2:1:4) (mg/ml). The antisera obtained were titrated both by complement fixation and by passive agglutination.
B, Zalc   +3 more
openaire   +2 more sources

Glucosylceramide in Humans

2010
Glucosylceramide has a unique and often ambiguous role in mammalian cells. Activation of glucosylceramide synthase, the enzyme that places a glucosyl moiety onto ceramide, is the first pathway-committed step to the production of more complex glycosphingolipids such as lactosylceramide and gangliosides.
Maria C, Messner, Myles C, Cabot
openaire   +2 more sources

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