The present invention provides a method of treating lysosomal storage
diseases such as Hurler syndrome and Batten disease in individuals in
need of such treatment, comprising the step of administering to said
individuals a therapeutically effective dose of an aminoglycoside. In
addition, this method may further comprise treating the individual with
enzyme replacement therapy. Furthermore, the present invention provides
method of pharmacologically suppressing premature stop mutations in an
individual with these lysosomal storage diseases, comprising the step of
administering to said individual a pharmacologically effective dose of an
aminoglycoside.