Figure 2.

Enzyme activities in brain cerebrum and cerebellum. Two pieces of the brain cortex and one piece from the left cerebellum dissected from perfused animals were tested for IDUA activity in triplicate. Data are generated from 5–12 animals in each group. Undetectable level of IDUA activity (<0.003 U/mg) was found in brain and cerebellum of untreated MPS I mice. IDUA activities in normal heterozygous mice were 4.3 ± 0.5 U/mg in cerebrum and 3.3 ± 0.4 U/mg in cerebellum. *P ≤0.05; **P < 0.001; NS, no significance. IDUA, α-l-iduronidase; MPS I, mucopolysaccharidosis type I.