Following is the structural formula of ganglioside GM, a macromolecular gly- colipid (meaning that it contains lipid and monosaccharide units joined by glycosidic bonds). (b) Но CH,OH (d) HO CH,OH (f) NH CH,OH OH (a) C=0- OCH,CHCHCH=CH(CH,)12CH3 OH ОН Но. ČH3 (c) NHC(CH,) 16CH3 (e) НО ОН OH НООС NH HO C=O CH3 Ganglioside GM or Tay-Sachs ganglioside In normal cells, this and other gangliosides are synthesized continuously and degraded by lysosomes, which are cell organelles containing digestive enzymes. If pathways for the degradation of gangliosides are inhibited, the gangliosides accumulate in the cen- tral nervous system, causing all sorts of life-threatening consequences. In inherited diseases of ganglioside metabolism, death usually occurs at an early age. Diseases of ganglioside metabolism include Gaucher's disease, Niemann-Pick disease, and Tay- Sachs disease. Tay-Sachs disease is a hereditary defect that is transmitted as an au- tosomal recessive gene. The concentration of ganglioside GM, is abnormally high in this disease because the enzyme responsible for catalyzing the hydrolysis of glycosidic bond (b) is absent.

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Metabolic pathways

Metabolism is defined as the group of life-supporting, enzyme-mediated chemical reactions in organisms. A metabolic pathway is a set of these chemical reactions that involve anabolism (synthesis of complex biomolecules) and catabolism (breakdown of biomolecules into small molecules) that occur inside a cell. Metabolites are the reacting substances, products, and intermediate substances of an enzymatic process that are altered by a series of chemical events catalyzed by enzymes.