bs-11756R-A488

DATASHEET

Host: Rabbit

Target Protein: SGSH/Sulphamidase

Immunogen Range: 301-388/502

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 6448

Swiss Prot: P51688

Source: KLH conjugated synthetic peptide derived from human Sulphamidase

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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

Background:

Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.

Conjugation: ALEXA FLUOR® 488

Excitation/ Emission: 490nm/525nm

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 55

Cross Reactive Species: Rat

Predicted Cross Reactive Species: Human
Mouse
Dog

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