SGSH Rabbit Polyclonal Antibody
Other products for "SGSH"
Specifications
Product Data | |
Applications | IHC, WB |
Recommended Dilution | IHC:1:100-1:1000; WB:1:1000-1:10000 |
Reactivities | Human |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | Recombinant fragment corresponding to a region within amino acids 318 and 496 of SGSH (Uniprot ID#P51688) |
Formulation | 0.1M Tris, 0.1M Glycine, 10% Glycerol (pH7). 0.01% Thimerosal was added as a preservative. |
Concentration | lot specific |
Purification | Purified by antigen-affinity chromatography. |
Conjugation | Unconjugated |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Predicted Protein Size | 57 kDa |
Gene Name | N-sulfoglucosamine sulfohydrolase |
Database Link | |
Background | 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. [provided by RefSeq] |
Synonyms | HSS; MPS3A; SFMD |
Note | Seq homology of immunogen across species: mouse (81%), cow (81%), Dog (82%) |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Glycosaminoglycan degradation, Lysosome, Metabolic pathways |
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