<prompt> The deficient enzyme in Niemann-Pick disease is:b-GlucosidaseSphingomyelinaseb-GalactosidaseCerebrosidase  <response>: Sphingomyelinase: Ref: 206-LP, 203-HP 56- R.S.7thNIEMANN-PICK DISEASE: - Sphingomyelinase deficiency Enlarged liver and spleen filled with lipid Severe mental retardation and neurodegeneration Death in early childhood. Tay-sachs disease GM2-gangliosidosis: - hexosaaminidase A GMi Gangliosidosis - b galactosidase Metachromic Leukodystrophy MLD: - Aryl sulfatase A Fabrys disease - a - Galactosidase Krabbes disease - b Galactosidase Gauchers disease - b - Glucosidase Farbers disease - Ceramide 