Species: | Rabbit |
Applications: | WB IHC IF |
Immunogen Range: | KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase |
Clonality: | Polyclonal Antibody |
Isotype: | IgG |
GENE ID: | 2799 |
Swiss Prot: | |
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. |
Purification: | Purified by Protein A. |
Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
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. |
Caculated MW: | / |
Observed MW: | Refer to Figures |
Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
Reacitivity: | Human, Mouse, Rat |