{"product_id":"es5825","title":"Factor I rabbit pAb","description":"\u003cstrong\u003eApplications:\u003c\/strong\u003e WB;ELISA\u003cbr\u003e\u003cstrong\u003eReactivity:\u003c\/strong\u003e Human;Rat;Mouse;\u003cbr\u003e\u003cstrong\u003eSource:\u003c\/strong\u003e Rabbit\u003cbr\u003e\u003cstrong\u003eDilution:\u003c\/strong\u003e IHC: 1\/500 - 1\/2000. ELISA: 1\/10000. Not yet tested in other applications.\u003cbr\u003e\u003cstrong\u003eImmunogen:\u003c\/strong\u003e The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490\u003cbr\u003e\u003cstrong\u003eStorage_stability:\u003c\/strong\u003e -20°C\/1 year\u003cbr\u003e\u003cstrong\u003eClonality:\u003c\/strong\u003e Polyclonal\u003cbr\u003e\u003cstrong\u003eIsotype:\u003c\/strong\u003e IgG\u003cbr\u003e\u003cstrong\u003eConcentration:\u003c\/strong\u003e 1 mg\/ml\u003cbr\u003e\u003cstrong\u003eObserved_band(KD):\u003c\/strong\u003e Full lenth:66kD, heavy chain: 50-58kD\u003cbr\u003e\u003cstrong\u003eHuman_gene_id:\u003c\/strong\u003e 3426\u003cbr\u003e\u003cstrong\u003eHuman_swiss_prot_no:\u003c\/strong\u003e P05156\u003cbr\u003e\u003cstrong\u003eSubcellular_location:\u003c\/strong\u003e Secreted, extracellular space. Secreted .\u003cbr\u003e\u003cstrong\u003eOther_name:\u003c\/strong\u003e CFI; IF; Complement factor I; C3B\/C4B inactivator\u003cbr\u003e\u003cstrong\u003eBackground:\u003c\/strong\u003e This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by Ref","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":50411404394776,"sku":"ES5825","price":250.0,"currency_code":"USD","in_stock":true}],"url":"https:\/\/danabiosci.com\/products\/es5825","provider":"Dana Bioscience","version":"1.0","type":"link"}