bs-13479R-Cy7

DATASHEET

Host: Rabbit

Target Protein: Glucosamine 6 sulfatase

Immunogen Range: 1-100/552

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 2799

Source: KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

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.

Conjugation: Cy7

Excitation/ Emission: 743nm/767nm

Size: 100ul

Concentration: 1ug/ul

Applications: IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 58

Predicted Cross Reactive Species: Human
Mouse
Rat
Cow
Horse
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.