SGSH Antibody / N-sulphoglucosamine sulphohydrolase / HSS
N-sulphoglucosamine sulphohydrolase is an enzyme that in humans is encoded by the SGSH gene. 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.
Product Specifications
CAS Number
9007-83-4
Specifications
Western blot: 0.5-1 µg/mL, Immunohistochemistry (FFPE) : 2-5 µg/mL, Flow cytometry: 1-3ug/million cells, Direct ELISA: 0.1-0.5 µg/mL
UniProt
P51688
Host
Rabbit
Reactivity
Human, Mouse, Rat
Immunogen
E. coli-derived recombinant human protein (amino acids R206-L502) was used as the immunogen for the SGSH antibody.
Clonality
Polyclonal
Isotype
IgG
Applications
WB, IHC-P, FACS, Direct ELISA
Purity
Antigen affinity purified
Format
Antigen affinity purified
Buffer
Lyophilized from 1X PBS with 2% Trehalose
Reconstitution
Limitations
This SGSH antibody is available for research use only.
Storage Conditions
Formulation
0.5 mg/mL if reconstituted with 0.2ml sterile DI water
Applications Notes
Optimal dilution of the SGSH antibody should be determined by the researcher.
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