Anti-GNS Antibody Picoband® Fluoro647 Conjugated
Product Specifications
Background
N-acetylglucosamine-6-sulfatase, also known as glucosamine (N-acetyl) -6-sulfatase, is an enzyme that in humans is encoded by the GNS gene. 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
N-acetylglucosamine-6-sulfatase; Glucosamine-6-sulfatase; G6S; GNS
Gene Name
GNS
Gene ID
2799
UniProt
P15586
Host
Rabbit
Reactivity
Human, Mouse, Rat
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
E. coli-derived human GNS recombinant protein (Position: W238-R355) .
Clonality
Polyclonal
Tissue Specificity
Found only in peripheral nervous system Schwann cells.
Applications
Flow Cytometry
Field of Research
Amino Acid Metabolism, Amino Acids, Cell Biology, Metabolic Signaling Pathways, Metabolism, Organelles, Pathways and Processes, Proteolysis/Ubiquitin, Proteolytic Enzymes, Signal Transduction, Subcellular Markers, Tags & Cell Markers
Purification
Immunogen affinity purified.
Form
Liquid
Function
Dopamine receptor responsible for neuronal signaling in the mesolimbic system of the brain, an area of the brain that regulates emotion and complex behavior. Activated by dopamine, but also by epinephrine and norepinephrine, and by numerous synthetic agonists and drugs. Agonist binding triggers signaling via G proteins that inhibit adenylyl cyclase. Modulates the circadian rhythm of contrast sensitivity by regulating the rhythmic expression of NPAS2 in the retinal ganglion cells.
References & Citations
1. Entrez Gene: Glucosamine (N-acetyl) -6-sulfatase. 2. Basner R, Kresse H, von Figura K (February 1979) . N-Acetylglucosamine-6-sulfate sulfatase from human urine. J. Biol. Chem. 254 (4) : 1151–8. 3. Kresse H, Fuchs W, Glössl J, Holtfrerich D, Gilberg W (December 1981) . N-acetylglucosamine-6-sulfate by human β-N-acetylhexosaminidase A. J. Biol. Chem. 256 (24) : 12926–32.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Specificity
No cross reactivity with other proteins.
Applications Notes
6
Gene Name Synonym
Glucosamine (N-acetyl) -6-sulfatase
Subcellular Location
Lysosome.
Protein Name
D (4) dopamine receptor
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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