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(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
色久悠悠婷婷综合在线亚洲 ,人人妻久久人人澡人人爽人人精品,免费无码百合真人片18禁
Rabbit Anti-Frataxin/BF350 Conjugated antibody (bs-9601R-BF350)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-9601R-BF350
英文名稱 Rabbit Anti-Frataxin/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的線粒體型共濟失調(diào)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(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  心血管  細胞生物  免疫學(xué)  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
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 1mg/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 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)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(quán)所有 2004-2026 www.ggggww.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)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(zhì)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
主站蜘蛛池模板: 亚洲国产精品久久久久久久 | 成年人免费视频网站 | 国产欧美日韩精品高清二区综合区 | 女人与zzzooooxxxx | av观看成片免费网站 | 国产高清精品二区 | 无码AV精品一区二区三区 | 成年美女黄网站色大免费全看 | 国产精品久久丫 | 亚洲Av秘?无码一区二区 | 韩日av在线播放 | 欧美乱大交xxxxx | 嗯快点别停舒服好爽受不了了 | 97国产一区 | 中文无码一区二区不卡AV | 男人扒开女人腿桶到爽免费 | 日本一区二区三区久久久久 | 被两个黑人玩得站不起来了 | 在线观看热码亚洲av每日更新 | 三级在线观看网站 | 黄色一级片在线免费观看 | 在线观看AV片永久免费 | 黄色二区 | 精品国产一区二区三区噜噜噜 | 韩国av永久免费 | 狠狠操狠狠摸 | 日韩黄色毛片 | 国产又硬又粗进去好爽A片软件 | 久久久久久综合网天天 | 无码国产福利av私拍 | 日日猛噜噜狠狠扒开双腿小说 | 国产精品国产三级国产普通话一 | 日韩第8页| aaaaaaa大片免费看 | 日韩美女在线观看 | 国产美女自卫慰视频福利 | 97国产人人| 男女做爰裸体猛烈吃奶摸视频 | 国产aⅴ精品一区二区三理论片 | 无码人妻aⅴ一区二区三区鲁大师 | 4444亚洲人成无码网在线观看 |