{"product_id":"recombinant-human-cystatin-b-bhp11003190","title":"Recombinant Human Cystatin-B","description":"\u003cp\u003e\u003cstrong\u003eRecombinant Human Cystatin-B\u003c\/strong\u003e is supplied as a recombinant protein for in vitro research use.\u003c\/p\u003e\n\u003ch3\u003eBackground\u003c\/h3\u003e\n\u003cp\u003eType 1 cystatins are also called stefins which function as intracellular thiol protease inhibitors. Cystatin-B protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. CSTB protein protects proteases leakage from lysosomes. Mutations in Stefin-B gene cause primary defects in patients with progressive myoclonic epilepsy (EPM1), a degenerative disease of the central nervous system. CSTB is overexpressed \u0026amp; elevated in the serum of HCC patients. Cystatin-B in vivo has a polymeric structure which is sensitive to the redox environment. Cystatin-B inhibits bone resorption by down-regulating intracellular cathepsin K activity despite increased osteoclast survival. Protein and mRNA levels of stefin B are significantly lower in atypical benign meningiomas. Stefins-A \u0026amp; Stefin-B which belong to the type-1 Cystatins, are up-regulated in lung tumours and thus able to counteract harmful tumour-associated proteolytic activity. Human stefin-A \u0026amp; Stefin-B form amyloid fibrils. Copper binding by stefin-B reduces amyloid fibril formation. A number of alternatively spliced CSTB isoforms were recognized in patients with progressive myoclonus epilepsy. Decreased CSTB activity in EPM1 pathogenesis is controled by cathepsins through increased activity of cathepsin-S \u0026amp; cathepsin-L.\u003c\/p\u003e\n\u003ch3\u003eProduct format\u003c\/h3\u003e\n\u003cp\u003eProvided as a recombinant protein suitable for in vitro workflows such as binding studies, screening, and assay development. Refer to the specifications table for expression format and molecular properties.\u003c\/p\u003e","brand":"ProSpec-Tany TechnoGene Ltd","offers":[{"title":"5 ug","offer_id":53038105002349,"sku":"pro-609-5UG","price":60.0,"currency_code":"USD","in_stock":true},{"title":"20 ug","offer_id":53038390870381,"sku":"pro-609-20UG","price":145.0,"currency_code":"USD","in_stock":true},{"title":"1 mg","offer_id":53038390903149,"sku":"pro-609-1MG","price":3600.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/Prospecbio-cystatin-b-human-PRO-609.png?v=1782158245","url":"https:\/\/www.ebiohippo.com\/products\/recombinant-human-cystatin-b-bhp11003190","provider":"BioHippo","version":"1.0","type":"link"}