Reduced α-Gal A enzyme activity in Fabry fibroblast cells and Fabry mice tissues induced by serum from antibody positive patients with Fabry disease
T Ohashi, S Iizuka, H Ida, Y Eto - Molecular genetics and metabolism, 2008 - Elsevier
Fabry disease is a progressive, life-threatening lysosomal storage disorder which is
characterized by deficient activity of the lysosomal enzyme α-galactosidase A. Studies have
demonstrated that both enzyme preparations currently available for treatment of Fabry
disease (ie, agalsidase beta and agalsidase alfa) elicit immune responses in the majority of
patients which negatively influences the reduction of urinary globotriaosylceramide
concentration. In the current study, agalsidase beta antibodies were found to be associated …
characterized by deficient activity of the lysosomal enzyme α-galactosidase A. Studies have
demonstrated that both enzyme preparations currently available for treatment of Fabry
disease (ie, agalsidase beta and agalsidase alfa) elicit immune responses in the majority of
patients which negatively influences the reduction of urinary globotriaosylceramide
concentration. In the current study, agalsidase beta antibodies were found to be associated …
