FITC标记的铁蛋白轻链抗体

¥2980
LMAI Bio
中国/美国/欧洲
2021-07-27 13:39

上海联迈生物工程有限公司

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产品属性
供应商上海联迈生物工程有限公司
数量大量
靶点详见说明书
级别1
目录编号LM-6403R-FITC
克隆性多克隆
抗原来源Rabbit
保质期1年
抗体英文名Anti-Ferritin Light Chain/FITC
抗体名Anti-Ferritin Light Chain/FITC
标记物FITC标记
宿主Human, Mouse, Rat, Dog, Pig, Cow, Horse,
适应物种Human, Mouse, Rat, Dog, Pig, Cow, Horse,
免疫原详见说明书
亚型IGg
形态粉末、液体、冻干粉
应用范围IF=1:50-200
浓度1mg/ml
保存条件-20 °C
规格100ul
产品说明
FITC标记的铁蛋白轻链抗体
英文名称Anti-Ferritin Light Chain/FITC
中文名称FITC标记的铁蛋白轻链抗体
别 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.
规格价格100ul/2980元 购买 大包装/询价
说 明 书100ul
研究领域心血管 细胞生物 神经生物学 信号转导
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Dog, Pig, Cow, Horse,
产品应用IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量19-21kDa
性 状Lyophilized or Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Ferritin Light Chain
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
产品介绍background:
This gene encodes the light subunit of the ferritin protein. Ferritin is the major intracellular iron storage protein in prokaryotes and eukaryotes. It is composed of 24 subunits of the heavy and light ferritin chains. Variation in ferritin subunit composition may affect the rates of iron uptake and release in different tissues. A major function of ferritin is the storage of iron in a soluble and nontoxic state. Defects in this light chain ferritin gene are associated with several neurodegenerative diseases and hyperferritinemia-cataract syndrome. This gene has multiple pseudogenes. [provided by RefSeq, Jul 2008].

Function:
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.

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.

DISEASE:
Defects in FTL are the cause of hereditary hyperferritinemia-cataract syndrome (HHCS) [MIM:600886]. 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) [MIM:606159]; 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.

Similarity:
Belongs to the ferritin family. 
Contains 1 ferritin-like diiron domain.

Database links:
UniProtKB/Swiss-Prot: P02792

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.