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
蜜乳av网站,婷婷视频导航,免费av网站观看
首頁 > 產品中心 > 一抗 > 產品信息
FLNB Rabbit pAb (bs-13181R)  
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
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價

產品編號 bs-13181R
英文名稱 FLNB Rabbit pAb
中文名稱 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.  
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
克 隆 號
交叉反應 Human (predicted: Mouse,Rat,Rabbit,Sheep,Cow,Dog)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 278 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FLNB/Filamin 3: 1551-1650/2602 
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
產品介紹 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.

SWISS:
O75369

Gene ID:
2317

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



產品圖片
Sample:U251(Human)Cell Lysate at 40 ug Primary: Anti-FLNB(bs-13181R)at 1/300 dilution Secondary: IRDye800CW Goat Anti-RabbitIgG at 1/20000 dilution Predicted band size: 278kD Observed band size: 278kD
Sample:Hela (Human)Cell Lysate at 40 ug Primary: Anti-FLNB(bs-13181R)at 1/300 dilution Secondary: IRDye800CW Goat Anti-RabbitIgG at 1/20000 dilution Predicted band size: 278kD Observed band size: 278kD
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: 在线国产福利 | 日日干夜夜草 | 香港a毛片免费全部播放 | 日产精品一区二区 | 91视频.com | 国产精品v欧美精品v日韩精品v | 99国产精品久久久久久久夜 | 国产无遮挡裸体美女视频 | 国产r级福利在线观看 | 久久精品国产亚洲香蕉 | 99精品国产综合久久精品自在 | 欧洲精品成人免费视频在线观看 | 68精品国产免费久久久久久婷婷 | www.99热视频 | 老头玩小嫩奶陈晴晴老刘 | 一区二区三区免费视频网站 | 老司机免费视频一区二区三区 | 成人爽爽| 欧美黄色片网站 | 日韩视频在线免费观看 | 国产乱码精品一品二品 | 明星ai换脸二三区入口 | 国产第一页浮力影院草草影视 | 情侣黄网站大全免费看 | 欧美激情成人在线 | 四虎网站 | 国产在线精品一区二区中文 | 91精品欧美一区综合在线观看 | 欧美日韩视频免费 | 亚洲喷水 | 亚洲午夜久久久影院 | 人妻AV无码一区二区三区 | 久久精品国产精品亚洲蜜月 | av影片免费在线观看 | 国产玖玖在线视频 | 亚洲一区精品在线观看 | 国产高清在线精品二区 | 美国av毛片 | 国产精品一区二区免费不卡 | 国产乱码精品一区二区三区麻豆 | 国产第一页浮力影院草草影视 |