| Catalog No. | Size | Price (USD) |
| 10319-RP02-100 | 100 µL | $147.96 |
| 10319-RP02-200 | 200 µL | $251.64 |
| 10319-RP02-400 | 400 µL | $355.32 |
General Information
| Product name | Anti-GNS Antibody, Rabbit Polyclonal |
| Validated applications | ELISA (FAQ Protocol) |
| Species reactivity | Reacts with: Human |
| Specificity | Human GNS |
| Immunogen | Recombinant Human GNS protein (Catalog#10319-H08H) |
| Preparation | Produced in rabbits immunized with purified, recombinant Human GNS / G6S (rhGNS; Catalog#10319-H08H; NP_002067.1; Met 1-Leu 552). Total IgG was purified by Protein A affinity chromatography. |
| Source | Polyclonal Rabbit IgG |
| Purification | Protein A |
| Formulation | 0.2 μm filtered solution in PBS |
| Conjugate | Unconjugated |
| Form | Liquid |
| Shipping | This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. |
| Storage | This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles. |
Synonyms: Anti-G6S Antibody
Background Information
| Full Name | glucosamine (N-acetyl)-6-sulfatase |
| Description | Glucosamine (N-acetyl)-6-sulfatase (GNS), also known as G6S, a hydrolase, which is one of the enzymes involved in heparan sulfate catabolism leading to lysosomal storage. GNS is required for the catabolism of the glycosaminoglycans (GAG) including heparin, heparan sulfate, and keratan sulfate through the hydrolysis of the 6-sulfate group from the N-acetyl-D-glucosamine 6-sulfate units. Mucopolysaccharidosis type IIID (MPS IIID) is the least common of the four subtypes of Sanfilippo syndrome. It is caused by a deficiency of N-acetylglucosamine-6-sulphatase. A mutation in GNS resulting in MPS IIID indicates the potential utility of molecular diagnosis for this rare condition. As the least common type of the four subtypes of Sanfilippo syndrome, MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. |
| References |
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