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Background: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]
| Target | SGSH |
| Reactivity | Human |
| Applications | WB, IHC, IF, ICC |
| MW(Calculated) | |
| MW(Observed) | 55kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Modification | |
| Recommended Dilution Ratio | WB 1:1000-5000; IHC 1:100-300; ICC/IF 1:100-300 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#YS0004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Source | |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Purity | |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | PT0814R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Sequence | |
| Specificity | Endogenous |
| Gene Name | SGSH; HSS |
| Protein Name | N-sulphoglucosamine sulphohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase |
| Other Name | SGSH; / HSS; / N-sulphoglucosamine sulphohydrolase; / Sulfoglucosamine sulfamidase; / Sulphamidase |
| Species | Human |
| Gene ID-1 | 6448 |
| Uniprot | P51688 |
| Species.1 | |
| Gene ID-2 | |
| Uniprot.1 | |
| Species.2 | |
| Gene ID-3 | |
| Uniprot.2 | |
| Organism-4 | |
| Gene ID-4 | |
| SwissProt-4 | |
| Background | 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] |
| Cellular Localization | Lysosome. |
| Tissue Expression | |
| Signaling_pathway | Cellular Processes >> Transport and catabolism >> Lysosome |
| Research Areas | |
| Function | Catalyzes a step in lysosomal heparan sulfate degradation. {ECO:0000269|PubMed:15146460, ECO:0000269|PubMed:24816101, ECO:0000269|PubMed:7493035}. |
| RRID |
