SGSH is one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.1) Sleat, D.E., et al. Mol. Cell Proteomics 5(4):686-701(2006)
Immunogen
This SGSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 420-449 amino acids from the C-terminal region of human SGSH.
Western Blot at 1:4000 dilution + HepG2 whole cell lysate Lysates/proteins at 20 ug per lane.
Application Notes
For WB starting dilution is: 1:4000
Additional Information
Form
Liquid
Storage Instructions
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.