Product Name: Glucosamine 6 sulfatase Antibody
Applications: WB, IHC-P, IF(IHC-P)
Reactivity: Human, Mouse, Rat
Conjugation: Unconjugated
Host: Rabbit
Sourcr: KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase
Clonality: Polyclonal
CAS NO: 40246-10-4
Product: Dextrorotation nimorazole phosphate ester
Isotype: IgG
Concentration: 1ug/ul
Purification: Purified by Protein A.
Storage: Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for 12 months.
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.
PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/20828556?dopt=Abstract
