Iduronate 2-Sulfatase, Polyclonal Antibody

Anti -Iduronate 2-Sulfatase (IDS)

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

Chromosome: X; NC_000023.10 (148560295..148586884, complement). Location: Xq28

Polyclonal

IgG

Goat

Recognizes human IDS. Shows ~50% crossreactivity with recombinant mouse IDS.

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a lyophilized powder from PBS, pH 7.4, 5% trehalose. Reconstitute with 500ul sterile PBS.

Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile PBS. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Reconstituted product is stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. 

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.

Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to the sex-linked Mucopolysaccharidosis Type II, also known as Hunter Syndrome. Iduronate-2-sulfatase has a strong sequence homology with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase. A splice variant of this gene has been described.

Antibodies; Abs to Enzymes

ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP)

Suitable for use in ELISA, Western Blot and Immunoprecipitation.
Dilution: Western Blot: 0.1ug/ml
Immunoprecipitation: 25ug/ml detects IDS in conditioned cell culture medium spiked with recombinant human IDS.

19,460 Da[Similar Products]

iduronate 2-sulfatase

iduronate 2-sulfatase; idursulfase; OTTHUMP00000216437; OTTHUMP00000216438; OTTHUMP00000216439; iduronate 2-sulfatase 14 kDa chain; iduronate 2-sulfatase 42 kDa chain; alpha-L-iduronate sulfate sulfatase

Iduronate-2-sulfatase

O60597_HUMAN

Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to the sex-linked Mucopolysaccharidosis Type II, also known as Hunter Syndrome. Iduronate-2-sulfatase has a strong sequence similarity with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described. [provided by RefSeq]

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; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: lysosomal lumen

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

Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; chondroitin sulfate catabolic process; carbohydrate metabolic process; pathogenesis

Disease: Mucopolysaccharidosis, Type Ii

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