|Detection of Human Iduronate 2‑Sulfatase/IDS by Western Blot. Western blot shows lysates of human peripheral blood mononuclear cells (PBMCs). PVDF membrane was probed with 1 µg/mL of Goat Anti-Human Iduronate 2‑Sulfatase/IDS Antigen Affinity-purified Polyclonal Antibody (Catalog # AF2449) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # HAF017). A specific band was detected for Iduronate 2‑Sulfatase/IDS at approximately 42 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.|
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.
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