Human Iduronate 2-Sulfatase/IDS Antibody

As a member of the sulfatase family, IDS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and dermatan sulfate (2, 3). It hydrolyzes the 2-sulfate group of the L-iduronate 2-sulfate units of the GAG. The IDS deficiency results in mucopolysaccharidosis II (MPS II or Hunter syndrome), an X-linked inborn error leading to lysosomal accumulation of the GAG and its excretion in urine. MPS II has a wide spectrum of clinical manifestations ranging from mild to severe. The deduced amino acid sequence of human IDS consists of a signal peptide (residues 1 - 25), a pro peptide (residues 26 - 33) and a mature chain (residues 34 - 550) that may be further processed into the 42 kDa chain (residues 34 - 455) and the 14 kDa chain (residues 456 - 550) (1). Recombinant human IDS corresponds to the single chain and has sulfatase activity described above.