HUMAN ASPARTYLGLUCOSAMINIDASE - A BIOCHEMICAL AND IMMUNOCYTOCHEMICAL CHARACTERIZATION OF THE ENZYME IN NORMAL AND ASPARTYLGLUCOSAMINURIA FIBROBLASTS
Aspartylglucosaminidase (AGA, EC 3.5.1.26) is an essential enzyme in the degradation of asparagine-linked glycoproteins. In man, deficient activity of this enzyme leads to aspartylglucosaminuria (AGU), a recessively inherited lysosomal storage disease. Here we used affinity-purified polyclonal antibodies against the native AGA and its denatured subunits to establish the molecular structure and int