Western Blot (WB)
(Western blot analysis of Sulphamidase expression in HepG2 (A), mouse liver (B), mouse testis (C) whole cell lysates.)
Rabbit anti-Human Sulphamidase Polyclonal Antibody | anti-SGSH antibody
Anti-Sulphamidase Antibody
Western Blot (WB)
(Western blot analysis of Sulphamidase expression in HepG2 (A), mouse liver (B), mouse testis (C) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of Sulphamidase expression in HepG2 (A), mouse liver (B), mouse testis (C) whole cell lysates.)
Immunohistochemistry (IHC)
(Immunohistochemical analysis of Sulphamidase staining in human stomach formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.)
Immunohistochemistry (IHC)
(Immunohistochemical analysis of Sulphamidase staining in human stomach formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.)
NCBI and Uniprot Product Information
NCBI Description
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, Jul 2008]
Uniprot Description
SGSH: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A); also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. Belongs to the sulfatase family.
Protein type: Hydrolase; EC 3.10.1.1; Glycan Metabolism - glycosaminoglycan degradation
Chromosomal Location of Human Ortholog: 17q25.3
Cellular Component: lysosomal lumen
Molecular Function: catalytic activity; metal ion binding; N-sulfoglucosamine sulfohydrolase activity; sulfuric ester hydrolase activity
Biological Process: glycosaminoglycan catabolic process; proteoglycan metabolic process
Disease: Mucopolysaccharidosis, Type Iiia
Research Articles on SGSH
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Product Notes
The SGSH sgsh (Catalog #AAA8245754) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-Sulphamidase Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Sulphamidase can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). WB: 1/500 - 1/2000; IHC: 1/50 - 1/100. Researchers should empirically determine the suitability of the SGSH sgsh for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Sulphamidase, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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