The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 0.1 - 0.3 µg/ml. Predicted molecular weight: 62 kDa.
Use a concentration of 3.75 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
Liver, kidney, lung, and placenta.
Involvement in disease
Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2) [MIM:309900]; also known as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation.
Belongs to the sulfatase family.
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.