Immunocytochemical analysis of normal and acid maltase-deficient muscle cultures
A. F. Miranda, S. Shanske, A. P. Hays and S. DiMauro
Muscle cultures from patients with infantile and later-onset acid maltase
deficiency (AMD) and from unaffected controls were studied
immunocytochemically with anti-acid maltase (anti-AM) antibodies and
fluorescein-labeled goat anti-rabbit IgG second antibody. In control muscle
cells, an intense granular distribution of staining was seen, consistent
with lysosomal localization of AM. Cultured muscle cells from two patients
with infantile AMD (Pompe's disease) did not fluoresce, whereas cells from
two patients with AMD of later onset did fluoresce, showing a distribution
similar to that of controls.