GNS Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	Western blot: 1:50 - 1:100. ELISA: 1:1,000.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	KLH conjugated synthetic peptide selected from the Center region of human GNS
Specificity	This antibody detects GNS at Center, N189.
Formulation	PBS with 0.09% (W/V) sodium azide State: Liquid Ig fraction
Concentration	lot specific
Purification	Prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS
Conjugation	Unconjugated
Storage Condition	Store the antibody at 2 - 8 °C up to one month or (in aliquots) at -20 °C for longer. Avoid repeated freezing and thawing.
Gene Name	glucosamine (N-acetyl)-6-sulfatase
Database Link	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, 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	Glucosamine-6-sulfatase
Note	Molecular weight: 62082 Da
Reference Data
Protein Families	Druggable Genome, Transmembrane
Protein Pathways	Glycosaminoglycan degradation, Lysosome, Metabolic pathways

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