The present invention provides a highly purified recombinant human precursor
N-acetylgalactosamine-4-sulfatase and biologically active mutants, fragments and
analogs thereof as well as pharmaceutical formulations comprising highly purified
recombinant human precursor N-acetylgalactosamine-4-sulfatase. The invention also
provides methods for treating diseases caused all or in part by deficiencies in
human N-acetylgalactosamine-4-sulfatase including MPS VI and methods for producing
and purifying the recombinant precursor enzyme to a highly purified form.