GNS Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	The immunogen is a synthetic peptide directed towards the C terminal region of human GNS
Specificity	Expected reactivity: Cow, Dog, Goat, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish Homology: Cow: 100%; Dog: 93%; Goat: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Zebrafish: 85%
Formulation	Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. Note that this product is shipped as lyophilized powder to China customers.
Concentration	lot specific
Purification	Affinity Purified
Conjugation	Unconjugated
Storage Condition	For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
Predicted Protein Size	62kDa
Gene Name	glucosamine (N-acetyl)-6-sulfatase
Database Link	NP_002067 Entrez Gene 2799 Human UniProt ID P15586
Background	GNS is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparin sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder ucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.
Synonyms	G6S; Glucosamine-6-sulfatase; MGC21274; N-acetylglucosamine-6-sulfatase
Reference Data
Protein Families	Druggable Genome, Transmembrane
Protein Pathways	Glycosaminoglycan degradation, Lysosome, Metabolic pathways

*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.