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--产品介绍:Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation. Also plays a role in delivery of iron to cells. Mediates iron uptake in capsule cells of the developing kidney.TargetFunctionStores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation. Also plays a role in delivery of iron to cells. Mediates iron uptake in capsule cells of the developing kidney.Involvement in disease:Defects in FTL are the cause of hereditary hyperferritinemia-cataract syndrome (HHCS) . It is an autosomal dominant disease characterized by early-onset bilateral cataract. Affected patients have elevated level of circulating ferritin. HHCS is caused by mutations in the iron responsive element (IRE) of the FTL gene.Defects in FTL are the cause of neurodegeneration with brain iron accumulation type 3 (NBIA3) ; also known as adult-onset basal ganglia disease. It is a movement disorder with heterogeneous presentations starting in the fourth to sixth decade. It is characterized by a variety of neurological signs including parkinsonism, ataxia, corticospinal signs, mild nonprogressive cognitive deficit and episodic psychosis. It is linked with decreased serum ferritin levels.
(1mg/1ml铁蛋白抗体(轻链)Anti-Ferritin Light Chain,的来源为Rabbit,其相关产品:免疫组化常用试剂免疫印迹常用试剂各种标记二抗)
--铁蛋白抗体(轻链)的参数标准:
浓度:1mg/1ml
交叉反应:hu, mo, rat
产品类型:一抗
研究领域:心血管细胞生物神经生物学信号转导
蛋白分子量:predicted molecular weight: 19-21kDa
性状:Lyophilized or Liquid
免疫原:KLH conjugated synthetic peptide derived from hu Ferritin Light Chain
亚型:polyclonal
纯化方法:affinity purified by Protein A
储存液:Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用:WB=1:100-500ELISA=1:500-1000IP=1:20-100IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
保存条件:Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
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