| beta-d-glucuronidase | <enzyme> An enzyme catalyzing the hydrolysis of various beta-d-glucuronides, liberating free d-glucuronic acid and an alcohol; a deficiency of this enzyme is associated with Sly syndrome. Synonym: glusulase, glycuronidase. (05 Mar 2000) |
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| beta-d-glucuronidase deficiency | A rare deficiency of beta-d-glucuronidase; an autosomal recessive disorder with several allelic forms, characterised by abnormal mucopolysaccharide metabolism leading to progressive mental deterioration, splenic and hepatic enlargement, and dysostosis multiplex. Synonym: mucopolysaccharidase. (05 Mar 2000) |