Ferritin Light Chain铁蛋白轻链抗体

本公司经销Ferritin Light Chain，铁蛋白轻链抗体，克隆类型为polyclonal，宿主来源是Rabbit，Ferritin Light Chain铁蛋白轻链抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-6403R
英文名称：Anti-Ferritin Light Chain
中文名称：铁蛋白轻链抗体
其他名称：名Ferritin light polypeptide; Ferritin L chain; Ferritin L subunit; Ferritin light chain; Ferritin light polypeptide like 3; FRIL; FRIL_HUMAN; FTL; L apoferritin; MGC71996; NBIA 3; NBIA3.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 19-21kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, dog, hrs, cow, pig
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.Subunit : Oligomer of 24 subunits. There are two types of subunits: L （light） chain ａnd H （heavy） chain. The major chain can be light or heavy, depending on the species ａnd tissue type. The functional molecule forms a roughly spherical shell with a diameter of 12 nm ａnd contains a central cavity into which the insoluble mineral iron core is deposited. Iron enters the spherical protein shell through pores that are formed between subunits. Mutations leading to truncation or the addition of extra residues at the C-terminus interfere with normal pore formation ａnd with iron accumulation.Similarity : Belongs to the ferritin family. Contains 1 ferritin-like diiron domain.