[HTML][HTML] Lysosomal acid α-glucosidase consists of four different peptides processed from a single chain precursor

RJ Moreland, X Jin, XK Zhang, RW Decker… - Journal of Biological …, 2005 - ASBMB
Pompe's disease is caused by a deficiency of the lysosomal enzyme acid α-glucosidase
(GAA). GAA is synthesized as a 110-kDa precursor containing N-linked carbohydrates
modified with mannose 6-phosphate groups. Following trafficking to the lysosome,
presumably via the mannose 6-phosphate receptor, the 110-kDa precursor undergoes a
series of complex proteolytic and N-glycan processing events, yielding major species of 76
and 70 kDa. During a detailed characterization of human placental and recombinant human …