n. a marked deficiency in the enzyme P-gluconidase. Such deficiency prevents the metabolic breakdown of complex carbohydrates, resulting in the buildup of mucopolysaccharides (also called mucoplysaccharidosis). The condition manifest as an enlargement of the liver or spleen, bone malformrations, and mental retardation. See Sly Syndrome.
BETA-GLUCURONIDASE DEFICIENCY: "Beta glucoronidase deficiency is an inherited metabolic disease which traced back to a defect in chromosome 7's short arm."