Cell surface sialylation of glycoproteins and glycosphingolipids
in cultured metastatic variant RNA-virus transformed non-producer BALB/c 3T3 cell lines.
1990) Total synthesis of the tumor-associated Lex family of glycosphingolipids
Potent GCS inhibitors reduce glucosylceramide (GlcCer), the substrate of GBA and the storage material in Gaucher, and also higher glycosphingolipids
including gangliosides and globosides, the causative storage materials in Tay-Sachs disease, GM1 gangliosidosis and Fabry disease.
Recognition of bacterial glycosphingolipids
by natural killer T cells.
In all glycosphingolipids
containing a ceramide backbone, the sphingosine chain binds the F' pocket while the fatty acid occupies the A' pocket, exposing the sugar head to the TCR.
Jares-Erijman, Ophidiacerebrosides: Cytotoxic Glycosphingolipids
Containing a Novel Sphingosine from a Sea Star, J.
Deficiency of mammalian alpha-L- fucosidases has been proved to cause the fucosidosis which results in the lethal accumulation of glycoproteins and fucosylated glycosphingolipids
in the nervous system (Delves 1998).
Tumor-associated gangliosides are sialyl glycosphingolipids
present on the outer surface of the cell plasma membrane (23).
It is a genetic disorder of X-linked inheritance caused by deficiency of lysosomal enzyme a-galactosidase A resulting in progressive accumulation of glycosphingolipids
within different body cells.
9 It is a rare X-linked recessive disorder, with an incidence of about 1:40 000,10 caused by the mutation in a-galactosidase, a gene on long arm of X chromosome11 and subsequent deficiency of lysosomal hydrolase, alpha-galactosidase, which results in the progressive deposition of uncleaved neutral glycosphingolipids
, predominantly a-galactosyl-lactosyl ceramide (trihexosyl ceramide) within the lysosomes of endothelial, perithelial, smooth muscle cells, autonomic nervous system, kidneys, eyes and heart.
A group of glycosphingolipids
found in an invertebrate: Their structures and biological significance Mei SATAKE and Eishichi MIYAMOTO Communicated by Kunihiko SUZUKI, M.
In FD, reduced or absent activity of [alpha]-galactosidase A results in accumulation of globotriaosylceramide (GL-3) and related glycosphingolipids
in various tissues.