TREATMENT OF α-GALACTOSIDASE A DEFICIENCY

摘要

PROBLEM TO BE SOLVED: To provide a highly refined α-Gal A and various refining methods for the same; to provide an α-Gal A preparation having altered electric charges and a method for producing the same; to provide an α-Gal A preparation having circulatory half-life extended in a mammalian host and a method for producing the same; and to provide a method for dosing such an α-Gal A preparation to a subject and a dosage thereof.SOLUTION: There is provided a composition comprising a human α-Gal A preparation refined to a homogeneity of at least 99.5% when determined by SDS-PAGE or reverse-phase HPLC, wherein the preparation contains various α-Gal A glycoforms, has a specific activity of at least 3.0×10units/mg protein, and substantially contains no lectin.