[HTML][HTML] Catalytic Mechanism of Human α-Galactosidase 2
AI Guce, NE Clark, EN Salgado, DR Ivanen… - Journal of biological …, 2010 - ASBMB
The enzyme α-galactosidase (α-GAL, also known as α-GAL A; EC 3.2. 1.22) is responsible
for the breakdown of α-galactosides in the lysosome. Defects in human α-GAL lead to the
development of Fabry disease, a lysosomal storage disorder characterized by the buildup of
α-galactosylated substrates in the tissues. α-GAL is an active target of clinical research:
there are currently two treatment options for Fabry disease, recombinant enzyme
replacement therapy (approved in the United States in 2003) and pharmacological …
for the breakdown of α-galactosides in the lysosome. Defects in human α-GAL lead to the
development of Fabry disease, a lysosomal storage disorder characterized by the buildup of
α-galactosylated substrates in the tissues. α-GAL is an active target of clinical research:
there are currently two treatment options for Fabry disease, recombinant enzyme
replacement therapy (approved in the United States in 2003) and pharmacological …