Definitions for "Tay-Sachs Disease"
A rare hereditary disease affecting lipid metabolism in humans, due to a deficiency of hexosaminidase. It occurs in infants and children, and causes death before the onset of adulthood. It occurs most commonly of people of Jewish origin from easter Europe. It is characterized by accumulation of lipids in nervous tissue, causes a red spot on the retina, and eventual blindness and paralysis before death.
an enzyme deficiency of lipid metabolism inherited as a recessive; causes death in early childhood.
inherited, progressive neuronal degeneration of the central nervous system due to a deficient lysosomal enzyme; it causes excessive accumulations of a lipid called ganglioside