| Overview |
| bs-13479R-Cy3 |
| GNS Polyclonal Antibody, Cy3 Conjugated |
| IF(IHC-P), IF(IHC-F), IF(ICC) |
| Human, Mouse, Rat, Cow, Horse, Rabbit |
| Specifications |
| Cy3 |
| Rabbit |
| KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase |
| Polyclonal |
| #REF! |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target |
| 2799 |
| Cytoplasm |
| 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. |
| 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 |
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