Lipids

SPHINGOLIPIDOSISObjective: Illustration the disorders related to sphingolipids and glycolipids


Sphingolipidosis are lipid storage diseases, and are group ofinherited disorders of sphingolipidsand glycolipids due to geneticdeficiency of one or more oflysosomal hydrolyase enzymes involved in degradation ofthese types of lipids with a rangeof clinical features. The deficiency is usually in a single specific hydrolyase enzyme and subsequent accumulation of single sphingolipid which is a substrate for the deficient enzyme. This occur mainly in CNS where neurological abnormalities can lead to early death.