IDS (Alpha L iduronate Sulfate sulfatase, Alpha-L-iduronate Sulfate sulfatase, AW214631, Ids, IDS_HUMAN, Iduronate 2 sulfatase 14kD Chain, Iduronate 2 sulfatase 42kD Chain, Iduronate 2 sulfatase, Iduronate 2-sulfatase 14kD Chain, Iduronate sulfatase, Idursulfase, MPS2, RP23-29M4.1, SIDS)

IDS (Iduronate 2-sulfatase), also known as SIDS, is a 550 amino acid protein that localizes to the lysosome and belongs to the sulfatase family. Expressed in lung, liver, kidney and placenta, IDS uses calcium as a cofactor to catalyze the hydrolysis of select sulfate groups on dermatan sulfate, heparan sulfate and heparin and, via this catalytic activity, is essential for the lysosomal degradation of both dermatan and heparan sulfate. Defects in the gene encoding IDS are the cause of mucopolysaccharidosis type 2 (MPS2), more commonly known as Hunter syndrome, which is characterized by skeletal...