Pharmaceutical compositions containing protease and methods for the treatment of lysosomal storage diseases

摘要

The invention provides a method and compositions for treating a subject with a lysosomal storage disease such as gaucher disease, by administering to a subject with a lysosomal storage disease a therapeutically effective amount of composition comprising at least one of a protease or peptidase in an amount sufficient to ameliorate, reduce or improve at least one symptom of the disease. The invention also provides dietary supplements for subjects having lysosomal storage diseases.

主权项

1. A method for reducing splenomegaly and increasing energy in patients with Gaucher disease, which method comprises orally administering to an individual in need thereof an effective amount of a pharmaceutically acceptable composition comprising a blend of the proteolytic enzymes comprising
peptidase FPII comprising casomophinase having DPP-IV activity, obtained by surface fermentation of Aspergillus oryzae and having an activity of 25,000 hemoglobin units of tyrosine (HUT) , acid-stable protease manufactured from wheat bran culture of Aspergillus and having 25 spectrophotometric acid protease units, protease from the fermentation of Aspergillus oryzae, containing both endo and exo-peptidase activity and having an activity of 20,000 HUT, lactase from the fermentation of Aspergillus oryzae and having 1,000 acid lactase units; papain having 2,500,000 food chemical codex plant units; and I-lysine in an amount sufficient to reduce said splenomegaly and increase energy in said patients; and wherein said proteolytic enzyme blend has 54,000 hemoglobin units on a tyrosine basis, 130 units of aminopeptidase activity and 50 spectrophotometric acid protease units.