GNS Polyclonal Antibody, AbBy Fluor-488 Conjugated
Applications
Predicted Reactivity
| Overview | |
| Catalog # | bs-13479R-BF488 |
| Product Name | GNS Polyclonal Antibody, AbBy Fluor-488 Conjugated |
| Applications |
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Predicted Reactivity | Human, Mouse, Rat, Cow, Horse, Rabbit |
| Specifications | |
| Conjugation | AbBy Fluor-488 |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target | |
| Gene ID | 2799 |
| Subcellular location | Cytoplasm |
| Synonyms | 2610016K11Rik; AU042285; C87209; G6S; Glucosamine N-acetyl 6 sulfatase; Glucosamine 6 sulfatase; Glucosamine-6-sulfatase; GNS; GNS_HUMAN; MGC21274; N acetylglucosamine 6 sulfatase [Precursor]; N-acetylglucosamine-6-sulfatase; N28088. |
| Background | GNS is a 552 amino acid lysosomal enzyme that hydrolyzes the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate and heparan sulfate. A member of the sulfatase family, GNS assists in the catabolism of heparin, and binds calcium as a cofactor. GNS deficiency results in an autosomal recessive lysosomal storage disorder known as mucopolysaccharidosis type IIID (Sanfilippo D syndrome), which is characterized by mild somatic disease and severe degeneration of the central nervous system. Subject to post-translational internal peptidase cleavage, GNS is encoded by a gene mapping to human chromosome 12q14.2 and mouse chromosome 10 D2. |
| Application Dilution | |
| IF(IHC-P) | 1:50-200 |
| IF(IHC-F) | 1:50-200 |
| IF(ICC) | 1:50-200 |