They also offer antibodies to glycosphingolipids
and inhibitors of enzymes involved in glycosphingolipid
Inhibition of glycosphingolipid
biosynthesis: application to lysosomal storage disorders.
This pathology is responsible for the progressive neutral glycosphingolipid
depositions in the lysosomes of the blood vessel walls throughout the body leading a multi-systemic disease (1).
Glucosaminyl (N-Acetyl) transferase (GCNT) also includes glycosyltransferase, and GCNT2 was reported as a glycosphingolipid
biosynthesis-related gene in chickens .
The neurovisceral storage disorder, Niemann-Pick type C, sequesters cholesterol and glycosphingolipid
The B subunit pentamer component of the toxin binds to glycosphingolipid
globotriosylceramide (Gb3) receptors of absorptive villi and Paneth cells in the mammalian intestine, and globotetraosylceramide (Gb4) surface receptors in renal glomerular cells and brain endothelia (18, 21-23).
These disorders are characterized by a disrupted lysosomal defect of glycosphingolipid
, accumulating in the organelle with the respective glycoconjugates.
These variables are related to seven metabolic pathways, including TCA cycle, ubiquinone and other terpenoid quinone biosyntheses, N-glycan biosynthesis, other glycan degradation, glycosaminoglycan degradation, and glycosylphosphatidylinositol (GPI) anchor biosynthesis and glycosphingolipid
metabolism leads to systemic lysosomal globotriaosylceramide accumulation with multiorgan systemic involvement and complex clinical presentation: acroparesthesias, angiokeratoma, hypohidrosis, corneal and lenticular opacities, gastrointestinal and endocrine abnormalities, renal impairment, and neural and cardiovascular disease [1, 2].
The type III or adult form has a late onset between 3 and 30 years and appears as extrapyramidal disorders due to local deposition of glycosphingolipid
in the caudate nucleus.
The psychosine receptor is a receptor for the glycosphingolipid
psychosine (PSY) and several related glycosphingolipids
Salyan et al., "Structural characterization of x2 glycosphingolipid
, its extended form, and its sialosyl derivatives: accumulation associated with the rare blood group p phenotype," Biochemistry, vol.