This gene encodes 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.
Dilutions
WB 1:500 - 1:2000 IHC 1:50 - 1:100
Format
Calculated MW
56kDa
Observed MW
57kDa
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1).
Modification
Unmodified
Storage
-20°C
Images
Western blot analysis of extracts of various cell lines, using SGSH antibody (144-08148) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 10s.
Immunohistochemistry of paraffin-embedded human liver using SGSH antibody (144-08148) at dilution of 1:100 (40x lens).
Immunohistochemistry of paraffin-embedded human stomach using SGSH antibody (144-08148) at dilution of 1:100 (40x lens).
Expiration:
12 months from the date of shipment when stored properly.
