超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

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  關于我們  聯系我們
色噜噜狠狠爱综合视频 ,国产精品扒开腿做爽爽的视频,亚洲第一极品精品无码
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-Frataxin/Gold Conjugated antibody (bs-9601R-Gold)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-9601R-Gold
英文名稱 Rabbit Anti-Frataxin/Gold Conjugated antibody
中文名稱 膠體金標記的線粒體型共濟失調蛋白抗體
別    名 X25; CyaY; d-FXN; FA antibody FARR; Frataxin mature form; Frataxin(81-210); FRDA; FRDA_HUMAN; Friedreich ataxia protein; FXN; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; MSF01; MSF31; MSF42.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  心血管  細胞生物  免疫學  神經生物學  信號轉導  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 19kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Frataxin
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 background:
Friedreich ataxia is a progressive neurodegenerative disorder caused by loss of function mutations in the frataxin gene. The human frataxin gene maps to chromosome 9q13.The frataxin gene encodes a mitochondrial protein of the same name. Frataxin assembles into a stable homopolymer with iron-binding capabilities. When expressed in E. Coli human frataxin binds iron atoms at a rate of 10 iron atoms per 1 molecule of the frataxin polymer. Thus, frataxin appears to function in some capacity for iron-storage for the mitochondria. Frataxin may also function as an activator of oxidative phosphorylation to increase mitochondrial membrane potential and elevate cellular ATP. Frataxin is expressed in tissues with high metabolic activity including heart, liver and brown fat.

Function:
Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.

Subunit:
Belongs to the frataxin family.

Subcellular Location:
Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.

Tissue Specificity:
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.

Post-translational modifications:
Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure.

DISEASE:
Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. [MISCELLANEOUS] The unusual migration profile of mature frataxin on SDS-PAGE due to its acidic N-terminus most likely contributed to conflicting reports for the N-terminus of the mature protein. Unlike prokaryotic and yeast frataxin homologs, which self-assemble at high iron concentrations, oligomerization of human frataxin is not induced by iron. The existence of a specialized mitochondrial ferritin in mammalia (FTMT) is suggesting that iron storage would be redundant function, at least in mammalian mitochondria.

Similarity:
Belongs to the frataxin family.

Database links:

Entrez Gene: 2395 Human

Omim: 606829 Human

SwissProt: Q16595 Human

Unigene: 20685 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.ggggww.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         京公網安備110107000727號
主站蜘蛛池模板: av在线中文播放 | 久久亚洲综合精品99国产 | 亚洲精品一卡二卡三卡四卡2021 | 日本三级吃奶头添泬播放 | 国产特级毛片AAAAAA高潮流水 | 国产成人久久av免费高清密臂 | 一级片黄色免费 | 亚洲乱码国产乱码精品精的特点 | 亚洲视频中文字幕在线 | 毛片av在线播放 | wwwww黄色| 久热精品视频在线免费观看 | 特级做a爰片毛片免费看1o8 | 免费av中文字幕 | 日韩精品久久久免费观看四虎 | 欧美亚洲另类在线视频 | 97欧美一乱一性一交一视频 | 久久99精品久久久秒播软件优势 | 亚洲国产欧美中文手机在线 | 亚洲激精日韩激精欧美精品 | 国产亚洲日本精品无码 | 日韩av在线网 | 国产自创无码av情景剧 | 酒店大战丝袜高跟鞋人妻 | 国产精品一区三区在线观看 | 韩国日本中文字幕 | 久久久精品蜜桃 | 成年女人永久免费观看视频 | 日韩v亚洲v欧美v精品综合 | 亚洲精品中文字幕乱码三区91 | 成人午夜av国产传媒 | 欧美性色黄大片 | 国产美女网站视频 | 日韩αv在线 | 欧美第一在线视频 | 强●媚薬レズ狂いatid543 | 国产精品成人一区无码 | 两个人免费视频www 国产精品偷窥熟女精品视频 | 国产精品久久久久久亚洲色 | 青青草免费在线观看 | 免费亚洲精品视频 |