Enzyme deficiency in Hurler syndrome?: (A) Iduronate sulfatase, (B) a-l-Iduronidase, (C) b-Galactosidase, (D) Galactosamine 6-sulfatase
Answer is B. Disease name Abbretion Enzyme Defective GaG(s) affected Symptoms Hurler syndrome MPS I a-l-Iduronidase Dermatan sulfate, Heparan sulfate Mental retardation, coarse facial features, hepatosplenomegaly, cloudy cornea Reference: Harper; 30th edition; Page no:639; Table: 50-8