IDS, Polyclonal Antibody

IDS; iduronate 2-sulfatase (Hunter syndrome); MPS2; SIDS; iduronate-2-sulfatase; IDS antibody; iduronate 2-sulfatase (Hunter syndrome) antibody; MPS2 antibody; SIDS antibody; iduronate-2-sulfatase antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-IDS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Peptide ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 16000.
Immunohistochemistry: In paraffin embedded Human Placenta shows lysosomal staining of trophoblasts. Recommended concentration, 3-5ug/ml.
Western Blot: Approx. 60kDa band observed in Human Liver lysates (calculated MW of 61.9kDa according to NP_000193.1). Recommended concentration: 0.1-0.3ug/ml.

(0.1ug/ml) staining of Human Liver lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

(3.8ug/ml) staining of paraffin embedded Human Placenta. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

34,893 Da

iduronate 2-sulfatase

iduronate 2-sulfatase

Iduronate 2-sulfatase

SIDS; Idursulfase  [Similar Products]

IDS_HUMAN

This gene encodes a member of the sulfatase family of proteins. The encoded preproprotein is proteolytically processed to generate two polypeptide chains. This enzyme is involved in the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease mucopolysaccharidosis type II, also known as Hunter syndrome. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]

IDS: Required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2); 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 *****hood, with attenuated somatic complications and often without mental retardation. Belongs to the sulfatase family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.1.6.13; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: lysosomal lumen

Molecular Function: iduronate-2-sulfatase activity; metal ion binding

Biological Process: chondroitin sulfate catabolic process; glycosaminoglycan catabolic process

Disease: Mucopolysaccharidosis, Type Ii

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