Goat anti-Human Iduronate 2-Sulfatase Polyclonal Antibody | anti-IDS antibody
Iduronate 2-Sulfatase (IDS) (Biotin)
Purified by immunoaffinity chromatography
Purified by immunoaffinity chromatography
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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 adulthood, 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: Glycan Metabolism - glycosaminoglycan degradation; EC 3.1.6.13; Hydrolase
Chromosomal Location of Human Ortholog: Xq28
Cellular Component: lysosomal lumen
Molecular Function: iduronate-2-sulfatase activity; metal ion binding
Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; chondroitin sulfate catabolic process; pathogenesis
Disease: Mucopolysaccharidosis, Type Ii
Research Articles on IDS
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Product Notes
The IDS ids (Catalog #AAA614506) is an Antibody produced from Goat and is intended for research purposes only. The product is available for immediate purchase. The Iduronate 2-Sulfatase (IDS) (Biotin) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Iduronate 2-Sulfatase can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Suitable for use in Western Blot.,, Recommended Dilutions:, Western Blot: 0.1-0.2ug/ml. The detection limit is ~20ng/lane under reducing and non-reducing conditions., Optimal dilutions to be determined by the researcher.,, Storage and Stability:, Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile TBS, 0.1% BSA. 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. . Researchers should empirically determine the suitability of the IDS ids for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Iduronate 2-Sulfatase, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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