APA Dictionary of Psychology

deficiency of the enzyme β-glucuronidase, which is involved in the breakdown of complex carbohydrates (see mucopolysaccharidosis). This deficiency is characterized by enlarged liver or spleen, dermatan sulphate in the urine, bone malformations, white-cell inclusions, and intellectual developmental disorder. The condition occurs because of mutations in the GUSB gene in the short arm of chromosome 7. Also called mucopolysaccharidosis Type VII; Sly syndrome.