catalog number :
MBS8508375
products full name :
SGSH Antibody (Rabbit Polyclonal)
products short name :
[SGSH]
other names :
[Homo sapiens N-sulfoglucosamine sulfohydrolase, mRNA; N-sulphoglucosamine sulphohydrolase; N-sulphoglucosamine sulphohydrolase; N-sulfoglucosamine sulfohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase]
products gene name :
[SGSH]
other gene names :
[SGSH; SGSH; HSS; SFMD; MPS3A; HSS]
reactivity :
Human. Predicted: Rat, Mouse
form :
Purified by protein affinity column containing 1% BSA and 0.02% NaN3
storage stability :
Already reconstituted in 200ul sterile H2O. The antibody is stable for at least one year when stored at-20 degree C after reconstitution. Avoid repeated freeze/defrost cycles.
tested application :
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
app notes :
ELISA: 1:20,000-1:80,000. WB: 1:200-1:1,000. IHC: 1:100-1:500
products description :
Rabbit polyclonal SGSH(1) antibody was raised against a recombinate human SGSH protein 330-502aa (BC047318).
ncbi mol weight :
56,695 Da
ncbi pathways :
Disease Pathway (530764); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (645297); HS-GAG Degradation Pathway (645307); Heparan Sulfate Degradation Pathway (413377); Heparan Sulfate Degradation Pathway (468270); Heparan Sulfate/heparin (HS-GAG) Metabolism Pathway (645304); Lysosome Pathway (99052); Lysosome Pathway (96865)
ncbi summary :
This gene encodes the enzyme sulfamidase; one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with the lysosomal storage disease mucopolysaccaridosis IIIA, also known as Sanfilippo syndrome A, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. [provided by RefSeq, Jun 2017]
uniprot summary :
Catalyzes a step in lysosomal heparan sulfate degradation.