{"product_id":"sgsh-antibody-cat-csb-pa021200esr1hu","title":"SGSH Antibody - Cat. #: CSB-PA021200ESR1HU","description":"\u003cp\u003e\u003cb\u003eSize \u003c\/b\u003e:50ul\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e:\u003c\/p\u003e\u003cp\u003e\u003cb\u003eAliases\u003c\/b\u003e:Heparan sulfate sulfatase antibody; Heparan sulphate sulphatase antibody; HSS antibody; MPS 3A antibody; MPS3 A antibody; MPS3A antibody; Mucopolysaccharidosis type IIIA antibody; N sulfoglucosamine sulfohydrolase (sulfamidase) antibody; N sulfoglucosamine sulfohydrolase antibody; N-sulphoglucosamine sulphohydrolase antibody; SFMD antibody; SGSH antibody; SPHM_HUMAN antibody; Sulfoglucosamine sulfamidase antibody; Sulphamidase antibody; Sulphoglucosamine sulphamidase antibody\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProduct Type\u003c\/b\u003e:Polyclonal Antibody\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen Species\u003c\/b\u003e:Homo sapiens (Human)\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniProt ID\u003c\/b\u003e:P51688\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e:Recombinant Human N-sulphoglucosamine sulphohydrolase protein (273-502AA)\u003c\/p\u003e\u003cp\u003e\u003cb\u003eRaised in\u003c\/b\u003e:Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecies Reactivity\u003c\/b\u003e:Human\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTested Applications\u003c\/b\u003e:ELISA, IHC; Recommended dilution: IHC:1:20-1:200\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e:SGSH(N-sulphoglucosamine sulphohydrolase) is also named as HSS, sulphamidase and belongs to the sulfatase family. Sulfamidase is synthesized as a 62 kDa precursor protein, which is modified with mannose 6-phosphate (M6P) residues, allowing their recognition by mannose-6-phosphate receptors in the Golgi complex and ensuring transport to the endosomal\/lysosomal system. It catalyzes the third step of degradation of glucosaminoglycans and is required for the degradation of heparan sulphate. Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A), also known as Sanfilippo syndrome A.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e:Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e:IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification Method\u003c\/b\u003e:Antigen Affinity Purified\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e:Non-conjugated\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBuffer\u003c\/b\u003e:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e:Liquid\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStroage\u003c\/b\u003e:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTarget Names\u003c\/b\u003e:SGSH\u003c\/p\u003e\u003cp\u003e\u003cb\u003eResearch Areas\u003c\/b\u003e:Neuroscience; Tags \u0026amp; Cell Markers; Signal transduction\u003c\/p\u003e","brand":"Gene Bio Systems","offers":[{"title":"Default Title","offer_id":40486092832868,"sku":"CSB-PA021200ESR1HU","price":331.95,"currency_code":"EUR","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/products\/no_image_default_image-jpeg_a2941ffa-d009-4f94-8a29-db9677eec831.jpg?v=1660140617","url":"https:\/\/www.genebiosystems.com\/en-fr\/products\/sgsh-antibody-cat-csb-pa021200esr1hu","provider":"GeneBio ","version":"1.0","type":"link"}