This invention provides compositions of active highly phosphorylated lysosomal sulfatase enzymes, their pharmaceutical compositions, methods of producing and purifying such lysosomal sulfatase enzymes and compositions and their use in the diagnosis, prophylaxis, or treatment of diseases and conditions, including particularly lysosomal storage diseases that are caused by, or associated with, a deficiency in the lysosomal sulfatase enzyme.本發明提供活性高磷酸化溶酶體硫酸酯酶酵素之組合物、其醫藥組合物、製備及純化此等溶酶體硫酸酯酶酵素及組合物之方法,及其在診斷、預防或治療疾病及病狀中之用途,該等疾病及病狀尤其包括缺乏溶酶體硫酸酯酶酵素所引起或相關之溶酶體儲存疾病。