Rabbit Anti-SGSH Polyclonal Antibody
DATASHEET
Host:Rabbit
Target Protein:SGSH
IR:Immunogen Range:301-388/502
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:6448
Swiss Prot:P51688
Source:KLH conjugated synthetic peptide derived from human Sulphamidase:301-388/502
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. 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.
Size:50ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Dog
仅供科研使用。不能用于人和动物治疗等其它临床诊断用！
关键字： SGSH;Heparan sulfate sulf;HSS;MPS 3A;MPS3A;SFMD;SGSH;SPHM_HUMAN;
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