Western Blot (WB)
(Anti-SGSH Antibody (C-Term) at 1:4000 dilution + HepG2 whole cell lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 57 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)
Rabbit anti-Human SGSH Polyclonal Antibody | anti-SGSH antibody
SGSH Antibody (C-Term)
Western Blot (WB)
(Anti-SGSH Antibody (C-Term) at 1:4000 dilution + HepG2 whole cell lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 57 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)
Western Blot (WB)
(Anti-SGSH Antibody (C-Term) at 1:4000 dilution + HepG2 whole cell lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 57 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)
?Bekri, S., et al. J. Inherit. Metab. Dis. 28(4):601-602(2005)
?Muschol, N., et al. Hum. Mutat. 23(6):559-566(2004)
?Zhang, H., et al. Nat. Biotechnol. 21(6):660-666(2003)
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: EC 3.10.1.1; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation
Chromosomal Location of Human Ortholog: 17q25.3
Cellular Component: lysosomal lumen
Molecular Function: N-sulfoglucosamine sulfohydrolase activity; sulfuric ester hydrolase activity; metal ion binding; catalytic activity
Biological Process: proteoglycan metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis
Disease: Mucopolysaccharidosis, Type Iiia
Research Articles on SGSH
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Product Notes
The SGSH sgsh (Catalog #AAA9211715) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 420-449. The SGSH Antibody (C-Term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's SGSH can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:4000. 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 "SGSH, 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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