View our list of available Coronavirus (COVID-19, SARS-CoV-2, 2019-nCOV) antibodies, recombinant proteins, ELISA Kits... Options Full Datasheet Printer Friendly Datasheet Prepare for Printing Full Datasheet Printer Friendly Datasheet Prepare for Printing Have Questions? Ask Us! Request A Quote Today! Add To Compare List anti-GNS/G6S antibody :: Rabbit anti-Human GNS/G6S Monoclonal Antibody Catalog # MBS2543929 Unit / Price 0.06 mL / $260 +1 FREE 8GB USB 0.12 mL / $380 +1 FREE 8GB USB 0.2 mL / $560 +1 FREE 8GB USB Product Name Product Info Accession #s Product Desc Diseases/Tissues/Pathways Applications References Product Name GNS/G6S, Monoclonal Antibody Full Product Name Rabbit anti Human GNS/G6S Monoclonal Antibody Product Gene Name anti-GNS/G6S antibody[Similar Products] Research Use Only For Research Use Only. Not for use in diagnostic procedures. TOP OMIM Z12173 mRNA Clonality Monoclonal Isotype IgG Host Rabbit Species Reactivity Human Immunogen Recombinant Human GNS protein Buffer 0.2 mum filtered solution in PBS with 5% trehalose Preparation and Storage This antibody can be stored at 2 degree C- 8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles. ISO Certification Manufactured in an ISO 9001:2015 Certified Laboratory. Other Notes Small volumes of anti-GNS/G6S antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply. TOP Related Product Information for anti-GNS/G6S antibody Glucosamine (N-acetyl)-6-sulfatase (GNS), also known as G6S, a hydrolase, which is one of the enzymes involved in heparan sulfate catabolism leading to lysosomal storage. GNS is required for the catabolism of the glycosaminoglycans (GAG) including heparin, heparan sulphate, and keratan sulphate through the hydrolysis of 6-sulfate group from the N-acetyl-D-glucosamine 6-sulfate units. Mucopolysaccharidosis type IIID (MPS IIID) is the least common of the four subtypes of Sanfilippo syndrome. It is caused by a deficiency of N-acetylglucosamine-6-sulphatase. A mutation in GNS resulting in MPS IIID indicates the potential utility of molecular identification for this rare condition. As the least common type of the four subtypes of Sanfilippo syndrome, MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. Applications Tested/Suitable for anti-GNS/G6S antibody Western Blot (WB), ELISA (EIA) Application Notes for anti-GNS/G6S antibody WB: 2-10 mug/mLELISA: 0.1-0.2 mug/mL. This antibody can be used at 0.1-0.2 mug/mL with the appropriate secondary reagents to detect Human GNS. The detection limit for Human GNS is approximately 0.00245 ng/well. TOP NCBI/Uniprot data below describe general gene information for GNS/G6S. It may not necessarily be applicable to this product. NCBI GI # 15214696 NCBI GeneID 2799 UniProt Secondary Accession # Q53F05; B4DYH8[Other Products] UniProt Related Accession # P15586[Other Products] Molecular Weight 59,989 Da TOP NCBI Official Full Name Glucosamine (N-acetyl)-6-sulfatase NCBI Official Synonym Full Names glucosamine (N-acetyl)-6-sulfatase NCBI Official Symbol GNS [Similar Products] NCBI Official Synonym Symbols G6S [Similar Products] NCBI Protein Information N-acetylglucosamine-6-sulfatase UniProt Protein Name N-acetylglucosamine-6-sulfatase UniProt Synonym Protein Names Glucosamine-6-sulfatase; G6S UniProt Gene Name GNS [Similar Products] UniProt Synonym Gene Names G6S [Similar Products] UniProt Entry Name GNS_HUMAN TOP NCBI Summary for GNS/G6S The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome. [provided by RefSeq, Jul 2008] TOP UniProt Comments for GNS/G6S GNS: Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D); also known as Sanfilippo D syndrome. MPS3D is a 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. Belongs to the sulfatase family.Protein type: EC 3.1.6.14; Glycan Metabolism - glycosaminoglycan degradation; HydrolaseChromosomal Location of Human Ortholog: 12q14Cellular Component: lysosomal lumenMolecular Function: N-acetylglucosamine-6-sulfatase activity; protein binding; sulfuric ester hydrolase activityBiological Process: glycosaminoglycan catabolic process; keratan sulfate catabolic processDisease: Mucopolysaccharidosis, Type Iiid Research Articles on GNS/G6S 1. 12 new patients and 15 novel mutations were identified in Mucopolysaccharidosis type IIID. TOP Precautions All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines. Disclaimer While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information. TOP Products associated with anti-GNS/G6S antibody Reference Product PubMed Publications ARSB antibody >2 publications with GNS/G6S and ARSB Pathways associated with anti-GNS/G6S antibody Products by Pathway Pathway Diagram Clathrin Derived Vesicle Budding Pathway antibodies Clathrin Derived Vesicle Budding Pathway Diagram Glycosaminoglycan Degradation Pathway antibodies Glycosaminoglycan Degradation Pathway Diagram Glycosaminoglycan Degradation Pathway antibodies Glycosaminoglycan Degradation Pathway Diagram Glycosaminoglycan Metabolism Pathway antibodies Glycosaminoglycan Metabolism Pathway Diagram Heparan Sulfate Degradation Pathway antibodies Heparan Sulfate Degradation Pathway Diagram Heparan Sulfate Degradation Pathway antibodies Heparan Sulfate Degradation Pathway Diagram Immune System Pathway antibodies Immune System Pathway Diagram Innate Immune System Pathway antibodies Innate Immune System Pathway Diagram Keratan Sulfate Degradation Pathway antibodies Keratan Sulfate Degradation Pathway Diagram Keratan Sulfate Degradation Pathway antibodies Keratan Sulfate Degradation Pathway Diagram Diseases associated with anti-GNS/G6S antibody Disease Name Pubmed Publications Kidney Diseases Antibodies >28 publications with GNS/G6S and Kidney Diseases Death Antibodies >14 publications with GNS/G6S and Death Hypertension Antibodies >13 publications with GNS/G6S and Hypertension Proteinuria Antibodies >12 publications with GNS/G6S and Proteinuria Inflammation Antibodies >9 publications with GNS/G6S and Inflammation Mucopolysaccharidosis III Antibodies >8 publications with GNS/G6S and Mucopolysaccharidosis III Pain Antibodies >6 publications with GNS/G6S and Pain Fever Antibodies >6 publications with GNS/G6S and Fever Fibrosis Antibodies >5 publications with GNS/G6S and Fibrosis Liver Diseases Antibodies >5 publications with GNS/G6S and Liver Diseases Organs/Tissues associated with anti-GNS/G6S antibody Organ/Tissue Name Pubmed Publications Blood Antibodies >55 publications with GNS/G6S and Blood Kidney Antibodies >30 publications with GNS/G6S and Kidney Brain Antibodies >29 publications with GNS/G6S and Brain Liver Antibodies >15 publications with GNS/G6S and Liver Nerve Antibodies >14 publications with GNS/G6S and Nerve Vascular Antibodies >13 publications with GNS/G6S and Vascular Bone Antibodies >12 publications with GNS/G6S and Bone Skin Antibodies >10 publications with GNS/G6S and Skin Muscle Antibodies >9 publications with GNS/G6S and Muscle Adrenal Gland Antibodies >9 publications with GNS/G6S and Adrenal Gland TOP