How are gangliosides formed?

With the exception of GM4, which is derived from galactosylceramide (GalCer), most gangliosides are synthesized from lactosylceramide (LacCer). First, a simple ganglioside, GM3, is synthesized by addition of a sialic acid to LacCer by CMP-sialic acid: LacCer α2–3 sialyltransferase (ST-I or GM3 synthase).

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What is sphingolipid storage disease?

Sphingolipid storage diseases are caused by defective catabolic activities in the endolysosomal system of the cells. Lysosomal accumulation occurs predominantly in cells and organs that have the highest rates of biosynthesis or uptake of the undegradeable sphingolipids and their precursors.

How are lipids produced?

Where do Lipids Come From? Excess carbohydrates in the diet are converted into triglycerides, which involves the synthesis of fatty acids from acetyl-CoA in a process known as lipogenesis, and takes place in the endoplasmic reticulum.

Are gangliosides polar?

Such highly polar, acidic, and relatively hydrophilic molecules have distinctive physical properties, which are essential for the vital functions of gangliosides in the membranes of the central nervous system and other tissues.

Are gangliosides acidic?

Gangliosides are highly complex acidic glycosphingolipids containing one or more sialic acid groups (N-acetyl or N-glycoloyl neuraminic acid).

Is ganglioside a lipid?

Gangliosides are complex lipids with a strong amphiphilic character due to the big saccharidic headgroup and the double-tailed hydrophobic moiety.

Where are cerebrosides found?

Cerebrosides are abundant in brain white matter and nerve myelin sheaths and they are present in small quantity within the cell membranes of other tissues. Brain white matter and, to a lesser extent, other tissues, also have lipids that contain sulfur.

What disease is associated with metabolic disorders of sphingolipids?

Introduction. Many experimental and clinical studies have described the role of sphingolipids in the pathogenesis of lifestyle diseases such as myocardial infarction, hypertension, stroke and diabetes mellitus. They are involved in the regulation of numerous cellular processes, including apoptosis [1].