Gangliosidoses

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Gangliosidoses

A group of often fatal inherited diseases marked by the accumulation of GANGLIOSIDES in LYSOSOMES secondary to enzymatic deficiency states. Gangliosidoses include TAY-SACHS DISEASE; GANGLIOSIDOSIS GM1; GANGLIOSIDOSES GM2; and SANDHOFF DISEASE; which share the infantile or childhood onset of CENTRAL NERVOUS SYSTEM deterioration. (Menkes, Textbook of Child Neurology, 5th ed, pp89-97)