α-l-iduronidase

α-L-Iduronidase is a key enzyme involved in the catabolism of glycosaminoglycans (GAG), heparin, and dermatan sulfate. Assessing the specific enzymatic activity of α-L-Iduronidase is a crucial parameter in the study of mucopolysaccharidosis type I (MPS).

4-Methylumbelliferyl-α-L-Iduronide is a common substrate for α-L-iduronidase catalytic activity.

Lepunafusp alfa (JR-171) is a blood-brain barrier-penetrating fusion protein comprising J-Brain Cargo and α-L-iduronidase (IDUA), a lysosomal enzyme. This compound effectively lowers levels of heparan sulfate (HS) and dermatan sulfate (DS) and is utilized in the study of mucopolysaccharidosis type I [1] [2].