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The antibody against SGSH was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against SGSH was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-01767A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | SGSH |
| Target Synonyms | HSS; SFMD; MPS3A; SGSH | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse lung | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | P51688 | Immunogen Sequence |
Uniprot Id
P51688
Target Species
Human
Target Name
SGSH
Target Full Name
N-sulphoglucosamine sulphohydrolase
Target Function
Catalyzes a step in lysosomal heparan sulfate degradation.
Target Involvement
Mucopolysaccharidosis 3A (MPS3A)
Target Subcellular Location
Lysosome.
Target Protein Families
Sulfatase family
Target Synonyms
Heparan sulfate sulfatase; Heparan sulphate sulphatase; HSS; MPS 3A; MPS3 A; MPS3A; Mucopolysaccharidosis type IIIA; N sulfoglucosamine sulfohydrolase (sulfamidase); N sulfoglucosamine sulfohydrolase; N-sulphoglucosamine sulphohydrolase; SFMD; SGSH; SPHM_HUMAN; Sulfoglucosamine sulfamidase; Sulphamidase; Sulphoglucosamine sulphamidase
Target 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.
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