国产成人福利久久久_2019色 亚洲 日韩 国产 在线_免费在线观看黄色视频亚洲不卡_综合自拍三级欧美_久久精品高清最新地址无码_成人网站在线下载_精品电影日韩亚洲_麻豆影院无码一级_欲妇的爆乳大肉臀小说_在线看亚洲十八禁网

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  人才招聘  關于我們  聯(lián)系我們
言情小说 君子以泽,梦入神机
首頁 > 產(chǎn)品中心 > 一抗 > 產(chǎn)品信息
Mouse Anti-Ferritin Light Chain  antibody (bsm-41189M)  
訂購熱線:400-901-9800
訂購郵箱:[email protected]
訂購QQ:  400-901-9800
技術支持:[email protected]
說明書: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
200ug(PBS only)/5600.00元
大包裝/詢價

產(chǎn)品編號 bsm-41189M
英文名稱 Mouse Anti-Ferritin Light Chain  antibody
中文名稱 鐵蛋白輕鏈單克隆抗體
別    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.  
研究領域 心血管  細胞生物  神經(jīng)生物學  信號轉導  
抗體來源 Mouse
克隆類型 Monoclonal
交叉反應 Human
產(chǎn)品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
細胞定位 細胞漿 細胞外基質 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 Recombinant human Ferritin Light Chain 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
P02792

Gene ID:
2512

Database links:

Entrez Gene: 2512 Human

Entrez Gene: 14325 Mouse

Entrez Gene: 29292 Rat

Omim: 134790 Human

SwissProt: P02792 Human

SwissProt: P29391 Mouse

SwissProt: P02793 Rat

Unigene: 433670 Human

Unigene: 713706 Human

Unigene: 30357 Mouse

Unigene: 431913 Mouse

Unigene: 1905 Rat



產(chǎn)品圖片
Sample: Lane 1: Recombinant human Ferritin Light Chain, His Primary: Anti-Ferritin Light Chain (bsm-41189M) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilution Predicted band size: 19-21 kDa Observed band size: 25 kDa
版權所有 2004-2026 ourmotor168.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號