MU-α-D-Galactopyranoside-Degrading Activity (nmol/h/mg protein) in Organs from Fabry Mice Treated with a Single Dose of the Modified NAGA, Agalsidase Beta, or Agalsidase Alfa
Fabry mice were injected with a single dose (2 mmol/h/kg body weight) of each of the enzymes and then sacrificed 1 hr after the administration. Wild-type, wild-type mice; Fabry, untreated Fabry mice; Modified NAGA, Fabry mice treated with the modified NAGA; Agalsidase beta, Fabry mice treated with agalsidase beta; and Agalsidase alfa, Fabry mice treated with agalsidase alfa. Error bars represent means ± SD (n = 3). The increases in the enzyme activity upon administration of the modified NAGA are greater in the kidneys (p = 0.005 and 0.003 in comparison to agalsidase beta and agalsidase alfa, respectively, t test) and heart (p = 0.001 and 0.003 in comparison to agalsidase beta and agalsidase alfa, respectively, t test) tissues than in the cases of the recombinant GLAs.