Glycolipids disorders

A common denominator finding in glycolipid disorders is the inability, in tysosomes, to break down particular glycolipids, resulting in glycolipid accumulation. Clinical findings commonly include mental retardation. 

V-hydroxyglycine ethyl ester affords nitrones in good yields (187). Several a-aryl-iV-methylnitrones have been produced in yields of 80% to 100% without solvents, in the presence of 3A molecular sieves (188). Using 4A molecular sieves, it was possible to carry out the synthesis of novel glycolipidic nitrones — potential antioxidant dmgs for neurodegenerative disorders (Scheme 2.22) (189). 

The inability to properly degrade membrane-associated gangliosides (glycolipids) can lead to severe psycho-motor developmental disorders, for example ... 

This micromembrane was also used to investigate the spontaneous formation of microdomains, when the distal lipid monolayer is made up of a lipid mixture. Microdomains are in the gel state when they consist primarily of glycolipids and sphingohpids, in a liquid-ordered state (so-called hpid rafts ) when they also contain cholesterol, and in a hquid-disordered state when they consist primarily... 

Moser, H. W., Sugita, M., Harbison, M. D., and Williams, M. Liver glycolipid steroid sulfates and steroid sulfatases in a form of metachromatic leukodystrophy associated with multiple sulfatase deficiencies. In Sphingolipids, Sphingolipidosis, and Allied Disorders (D. W. Volk, and S. M. Aronson, eds.), pp. 429-450, Plenum, New York, 1972. 

GM2 gangliosidosis is a family of autosomal recessive disorders characterized by accumulation of GM2 ganglioside and its related glycolipids in the neuronal lysosome. It comprises GM2 activator protein deficiency, Tay-Sachs and Sandhoff disease, the latter of which are caused by a deficiency in the a-subunit or B-subunit of B-hexosaminidase, respectively (Gravel et ah, 1995). 

Butters, T. D., Pharmacotherapeutic strategies using small molecules for the treatment of glycolipid lysosomal storage disorders. Expert Opin Pharmacother 8 (2007b) 427-435. 

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