IDUA hydrolyses Heparan sulfate chain(1)

An L-iduronic acid residue can be cleaved from either heparan sulfate or dermatan sulfate by the lysosomal enzyme alpha-L-iduronidase (IDUA) (Scott et al. 1991). Defects in IDUA are the cause of mucopolysaccharidosis type IH (MPS IH, Hurler syndrome, MIM:607014), mucopolysaccharidosis IH/S (MPSIH/S, HurlerScheie syndrome, MIM:607015) and mucopolysaccharidosis type IS (MPSIS, Scheie syndrome, MIM:607016) (LeeChen et al. 1999).

α-L-艾杜糖酸残基可由溶酶体酶α-L-艾杜糖苷酸酶(IDUA)从肝素硫酸盐或透明质酸硫酸盐中裂解而来(Scott 等人，1991年)。IDUA基因的缺陷是粘多糖病I型(Hurler综合征，MPS IH，MIM:607014)、粘多糖病I/S(Hurler-Scheie综合征，MPSIH/S，MIM:607015)以及粘多糖病I型(Scheie综合征，MPSIS，MIM:607016)的病因(Lee-Chen 等人，1999年)。