Table 2.

Investigation of lysosomal enzymes

Sr. No.	Enzyme	Substrate	Disorder
1	α-Iduronidase	4-mu-α-L-iduronide	Hurler (MPS-I)
2	β-Galactosamine-6-sulfatase	4-mu-β-Galactose-6-sulfate	Morquio (MPS-IVA)
3	β-D-Glucuronidase	4-mu-β-D-glucuronide	Sly (MPS-VII)
4	Sphingomyelinase	4-mu-A-D-mannopyranoside	NPD-A/B
5	β-D-Galactosidase	4-mu-β-D-galactopyranoside	GM1 gangliosidosis
6	β-Glucosidase	4-mu-β-D-glucopyranoside	Gaucher
7	Free and total N-acetylneuraminic acid	N-acetylneuraminic acid (NANA)	Infantile sialic acid storage disorder (ISSD)