{"product_id":"abcam-ab232666","title":"Abcam, ab232666, Anti-SGSH\/HSS antibody [EPR17312] - BSA and Azide free","description":"\u003cp\u003eSize: 100µg \/ 1mg\u003cbr\u003e\nRabbit Recombinant Monoclonal SGSH\/HSS antibody. Carrier free. Suitable for WB, ICC\/IF and reacts with Human samples. Cited in 1 publication.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:EPR17312,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:Yes,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nApplications:ICC\/IF, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:The exact immunogen used to generate this antibody is proprietary information.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nab232666 is the carrier-free version of\u003cbr\u003e\nab200346\u003cbr\u003e\nPatented technology\u003cbr\u003e\nOur RabMAb\u003cbr\u003e\ntechnology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to\u003cbr\u003e\nRabMAb® patents\u003cbr\u003e\nWhat are the advantages of a recombinant monoclonal antibody?\u003cbr\u003e\nThis product is a recombinant monoclonal antibody, which offers several advantages including:\u003cbr\u003e\n- High batch-to-batch consistency and reproducibility\u003cbr\u003e\n- Improved sensitivity and specificity\u003cbr\u003e\n- Long-term security of supply\u003cbr\u003e\n- Animal-free batch production\u003cbr\u003e\nFor more information, read more on\u003cbr\u003e\nrecombinant antibodies\u003cbr\u003e\nConjugation ready\u003cbr\u003e\nOur carrier-free antibodies are typically supplied in a PBS-only formulation, purified and free of BSA, sodium azide and glycerol. This conjugation-ready format is designed for use with fluorochromes, metal isotopes, oligonucleotides, and enzymes, which makes them ideal for antibody labelling, functional and cell-based assays, flow-based assays (e.g. mass cytometry) and Multiplex Imaging applications.\u003cbr\u003e\nUse our\u003cbr\u003e\nconjugation kits\u003cbr\u003e\nfor antibody conjugates that are ready-to-use in as little as 20 minutes with 1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.\u003cbr\u003e\nCompatibility\u003cbr\u003e\nThis product is compatible with the Maxpar\u003cbr\u003e\nAntibody Labeling Kit from Fluidigm, without the need for antibody preparation. Maxpar\u003cbr\u003e\nis a trademark of Fluidigm Canada Inc.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.2 - 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°C, Storage information-Do Not Freeze\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nThe SGSH protein also known as N-sulfoglucosamine sulfohydrolase or HSS is a lysosomal enzyme with a molecular weight of approximately 56 kDa. It is primarily expressed in tissues with high turnover of glycosaminoglycans especially in the liver and brain. This enzyme localizes inside the lysosomes and plays a significant role in the degradation process of heparan sulfate. SGSH expression is important for maintaining normal cell function particularly in tissues that require constant renewal of extracellular matrix components.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThe enzyme acts to cleave sulfate groups from N-sulfated glucosamine residues in heparan sulfate being an important component of the degradation pathway of glycosaminoglycan. SGSH works within a lysosomal degradation complex alongside other enzymes such as iduronate-2-sulfatase and heparan-alpha-glucosaminide N-acetyltransferase each responsible for different steps in the breakdown of complex carbohydrates. Together they facilitate the complete degradation of heparan sulfate into its basic monosaccharide components.\u003cbr\u003e\nPathways\u003cbr\u003e\nSGSH is integral to lysosomal degradation pathways functioning alongside proteins like alpha-L-iduronidase in the catabolic process of heparan sulfate. This breakdown pathway is essential for recycling cellular components and maintaining cellular homeostasis. The efficiencies and deficiencies in these pathways influence cellular functionality and have regulatory roles in broader metabolic circuits.\u003cbr\u003e\nMutations in the SGSH gene lead to Sanfilippo syndrome type IIIA also known as mucopolysaccharidosis type IIIA. This genetic disorder impairs the degradation of heparan sulfate causing an accumulation of glycosaminoglycans in cells leading to neurodegenerative conditions. In Sanfilippo syndrome type IIIA the SGSH deficiency has connections with related lysosomal storage disorders involving proteins like alpha-N-acetylglucosaminidase which further illustrate the importance of coordinated lysosomal activities.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46851845259433,"sku":"ab232666","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab232666","provider":"Iright","version":"1.0","type":"link"}