Which enzyme plays a crucial role in the regulation of glycosaminoglycan synthesis, and its deficiency is associated with a lysosomal storage disorder characterized by clouded corneas and skeletal abnormalities?

A Alpha-L-iduronidase ✓
B Iduronate-2-sulfatase
C N-acetylgalactosamine-6-sulfatase
D Glucocerebrosidase

Explanation

The enzyme alpha-L-iduronidase plays a crucial role in the regulation of glycosaminoglycan synthesis. A deficiency in this enzyme is associated with Hurler syndrome, a lysosomal storage disorder characterized by clouded corneas and skeletal abnormalities.

Mnemonic

_L-iduronidase = Lysosomal ID, HurLer = Hurler syndrome

Source

NCERT Class 12 Biochemistry
Enzymes in Disease Biochemistry Lysosomal Storage Disorders Hard MEDICAL Confidence: high