97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

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
7777精品伊人久久久大香线蕉超级流畅 ,三级黄色片在线,快色视频黄观看
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-FLNB/BF555 Conjugated antibody (bs-13181R-BF555)
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  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13181R-BF555
英文名稱 Rabbit Anti-FLNB/BF555 Conjugated antibody
中文名稱 BF555標記的細絲蛋白3抗體
別    名 ABP 278; ABP 280 homolog; ABP-278; ABP-280 homolog; ABP278; Actin binding like protein; Actin binding protein 278; Actin-binding-like protein; AOI; Beta filamin; Beta-filamin; DKFZp686A1668; DKFZp686O033; Fh1; Filamin 1 actin binding protein 280 like; Filamin 3; Filamin B; Filamin B beta actin binding protein 278; Filamin homolog 1; Filamin-3; Filamin-B; FLN B; FLN-B; FLN1L; FLN3; FLNB; FLNB_HUMAN; LRS1; SCT; TABP; TAP; Thyroid autoantigen; Truncated ABP; Truncated actin binding protein; Truncated actin-binding protein.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  神經生物學  信號轉導  結合蛋白  細胞分化  細胞骨架  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, Dog, Cow, Rabbit, Sheep, )
產品應用 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 278kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FLNB/Filamin 3
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.
產品介紹 background:
This gene encodes a member of the filamin family. The encoded protein interacts with glycoprotein Ib alpha as part of the process to repair vascular injuries. The platelet glycoprotein Ib complex includes glycoprotein Ib alpha, and it binds the actin cytoskeleton. Mutations in this gene have been found in several conditions: atelosteogenesis type 1 and type 3; boomerang dysplasia; autosomal dominant Larsen syndrome; and spondylocarpotarsal synostosis syndrome. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene. [provided by RefSeq, Nov 2009].

Function:
Connects cell membrane constituents to the actin cytoskeleton. May promote orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton. Interaction with FLNA may allow neuroblast migration from the ventricular zone into the cortical plate. Various interactions and localizations of isoforms affect myotube morphology and myogenesis. Isoform 6 accelerates muscle differentiation in vitro.

Subunit:
Homodimer. Isoform 1 interacts with FBLP1, FLNA, FLNC, GP1BA, INPPL1, ITGB1A, PSEN1 and PSEN2. Isoform 3 interacts with ITGB1A, ITGB1D, ITGB3 and ITGB6. Interacts with MYOT and MYOZ1. Interacts with HBV capsid protein.

Subcellular Location:
Isoform 1: Cytoplasm, cell cortex. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Note=In differentiating myotubes, isoform 1, isoform 2 and isoform 3 are localized diffusely throughout the cytoplasm with regions of enrichment at the longitudinal actin stress fiber. In differentiated tubes, isoform 1 is also detected within the Z-lines.
Isoform 2: Cytoplasm, cytoskeleton. Note=Predominantly localized at actin stress fibers.
Isoform 3: Cytoplasm, cytoskeleton. Note=Predominantly localized at actin stress fibers.
Isoform 6: Cytoplasm, cytoskeleton. Note=Polarized at the periphery of myotubes.

Tissue Specificity:
Ubiquitous. Isoform 1 and isoform 2 are expressed in placenta, bone marrow, brain, umbilical vein endothelial cells (HUVEC), retina and skeletal muscle. Isoform 1 is predominantly expressed in prostate, uterus, liver, thyroid, stomach, lymph node, small intestine, spleen, skeletal muscle, kidney, placenta, pancreas, heart, lung, platelets, endothelial cells, megakaryocytic and erythroleukemic cell lines. Isoform 2 is predominantly expressed in spinal cord, platelet and Daudi cells. Also expressed in thyroid adenoma, neurofibrillary tangles (NFT), senile plaques in the hippocampus and cerebral cortex in Alzheimer disease (AD). Isoform 3 and isoform 6 are expressed predominantly in lung, heart, skeletal muscle, testis, spleen, thymus and leukocytes. Isoform 4 and isoform 5 are expressed in heart.

Post-translational modifications:
ISGylation prevents ability to interact with the upstream activators of the JNK cascade and inhibits IFNA-induced JNK signaling.

DISEASE:
Note=Interaction with FLNA may compensate for dysfunctional FLNA homodimer in the periventricular nodular heterotopia (PVNH) disorder.
Defects in FLNB are the cause of atelosteogenesis type 1 (AO1) [MIM:108720]; also known as giant cell chondrodysplasia or spondylohumerofemoral hypoplasia. Atelosteogenesis are lethal short-limb skeletal dysplasias with vertebral abnormalities, disharmonious skeletal maturation, poorly modeled long bones and joint dislocations.
Defects in FLNB are the cause of atelosteogenesis type 3 (AO3) [MIM:108721]. Atelosteogenesis are short-limb lethal skeletal dysplasias with vertebral abnormalities, disharmonious skeletal maturation, poorly modeled long bones and joint dislocations. In AO3 recurrent respiratory insufficiency and/or infections usually result in early death.
Defects in FLNB are the cause of boomerang dysplasia (BOOMD) [MIM:112310]. This is a perinatal lethal osteochondrodysplasia characterized by absence or underossification of the limb bones and vertebre. Boomerang dysplasia is distinguished from atelosteogenesis on the basis of a more severe defect in mineralisation, with complete absence of ossification in some limb elements and vertebral segments.
Defects in FLNB are the cause of Larsen syndrome (LRS) [MIM:150250]. An osteochondrodysplasia characterized by large-joint dislocations and characteristic craniofacial abnormalities. The cardinal features of the condition are dislocations of the hip, knee and elbow joints, with equinovarus or equinovalgus foot deformities. Spatula-shaped fingers, most marked in the thumb, are also present. Craniofacial anomalies include hypertelorism, prominence of the forehead, a depressed nasal bridge, and a flattened midface. Cleft palate and short stature are often associated features. Spinal anomalies include scoliosis and cervical kyphosis. Hearing loss is a well-recognized complication. Defects in FLNB are the cause of spondylocarpotarsal synostosis syndrome (SCT) [MIM:272460]; also known as spondylocarpotarsal syndrome (SCT) or congenital synspondylism or vertebral fusion with carpal coalition or congenital scoliosis with unilateral unsegmented bar. The disorder is characterized by short stature and vertebral, carpal and tarsal fusions.

Similarity:
Belongs to the filamin family.
Contains 1 actin-binding domain.
Contains 2 CH (calponin-homology) domains.
Contains 24 filamin repeats.

Database links:

Entrez Gene: 2317 Human

Entrez Gene: 286940 Mouse

Entrez Gene: 306204 Rat

Omim: 603381 Human

SwissProt: O75369 Human

SwissProt: Q80X90 Mouse

Unigene: 476448 Human

Unigene: 489652 Mouse



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.kastlife.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: 色妞AV永久一区二区国产AV | 日韩一二区在线 | 国产女同2互磨高潮在线观看 | 亚洲a一区二区三区 | 在线观看黄色片 | 少妇被粗大的猛烈进出动态图片 | 国产精品午夜爆乳美女 | 亚洲激情第一页 | 好爽好大久久久级淫片毛片小说 | 日韩欧美的一区二区 | 精品中文一区 | 一道本一区二区三区 | 亚洲av禁18成人毛片一级 | 久久精品成人一区二区三区 | 精品无人码麻豆乱码1区2区 | 毛片一级在线 | 免费久久一级欧美 | 国产色噜噜噜91在线精品 | 一区二三区日韩精品 | 永久免费精品影视网站播放器 | 校园春色另类小说 | 91视频www. | 一本一本久久A久久综合精品蜜桃 | 在线观看91精品国产入口 | 欧美黑人粗暴多交高潮水最多 | 亚洲V国产V天堂A无码二区 | 久久精品亚洲男人的天堂 | 丁香五月欧美成人 | www.亚洲精品 | 久久久久久久人妻无码中文字幕爆 | 久久久99精品免费观看乱色 | 色黄啪啪网18以下勿进 | 欧美日韩一区二区精品 | 国产香蕉一区二区三区 | jjzzjjzz亚洲 | 2019年手机中文字幕 | 日本不卡免费一区二区三区综合久久 | 最新国产福利 | 在线看片免费人成视频影院看 | 2019中文字幕在线 | 在线不卡AV片免费观看 |