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
又爽又色禁片1000视频免费看,亚洲美女视频在线观看,99爱99
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-FHL1/PE-Cy7 Conjugated antibody (bs-4827R-PE-Cy7)
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-4827R-PE-Cy7
英文名稱 Rabbit Anti-FHL1/PE-Cy7 Conjugated antibody
中文名稱 PE-Cy7標記的骨骼肌蛋白FHL1抗體
別    名 bA535K18.1; FHL 1; FHL 1B; FHL-1; FHL1; FHL1 protein; FHL1_HUMAN; FHL1A; FHL1B; FLH1A; Four and a half LIM domains 1; Four and a half LIM domains protein 1; Four and a half Lin11 Isl 1 and Mec 3 domains 1; KYO T; LIM protein SLIMMER; MGC111107; RAM14-1; RBP associated molecule 14-1; Skeletal muscle LIM protein 1; Skeletal muscle LIM-protein 1; SLIM 1; SLIM; SLIM-1; SLIM1; SLIMMER; XMPMA.  
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  轉錄調節因子  鋅指蛋白  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, 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 35kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FHL1
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 four-and-a-half-LIM-only protein family. Family members contain two highly conserved, tandemly arranged, zinc finger domains with four highly conserved cysteines binding a zinc atom in each zinc finger. Expression of these family members occurs in a cell- and tissue-specific mode and these proteins are involved in many cellular processes. Mutations in this gene have been found in patients with Emery-Dreifuss muscular dystrophy. Multiple alternately spliced transcript variants which encode different protein isoforms have been described.[provided by RefSeq, Nov 2009]

Function:
May have an involvement in muscle development or hypertrophy.

Subcellular Location:
Isoform 1: Cytoplasm.
Isoform 3: Cytoplasm. Nucleus.
Isoform 2: Nucleus. Cytoplasm, cytosol. Note=Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.

Tissue Specificity:
Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.

DISEASE:
Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy (SPM) [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear.
Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies.
Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases.
Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (CO-RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717].

Similarity:
Contains 3 LIM zinc-binding domains.

Database links:

Entrez Gene: 509056 Cow

Entrez Gene: 2273 Human

Entrez Gene: 14199 Mouse

Entrez Gene: 25177 Rat

Omim: 300163 Human

SwissProt: Q13642 Human

SwissProt: P97447 Mouse

SwissProt: Q9WUH4 Rat

Unigene: 435369 Human

Unigene: 3126 Mouse

Unigene: 54261 Rat



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: 亚洲日韩中文第一精品 | 一级欧美一级日韩片免费观看 | a级片在线观看 | 午夜福利麻豆国产精品 | av资源站最稳定的资源站 | 日本黄色片免费观看 | 欧美jizz18hd性欧美 | 成熟丰满熟妇XXXXX丰满 | 黑人成人网 | 97激情 | 四川骚妇无套内射舔了更爽 | 日本a级影片 | 91·com| 日韩高清免费在线 | 老熟妇性老熟妇性色变态 | 精品成人免费一区二区不卡 | 亚洲a人 | 欧美精品综合A片在线观看 精品一区二区三区四 | 免费看91视频 | 一区二区三区在线视频播放 | 91老司机福利在线 | 午夜精品久久久久久久99 | 精品国产免费一区二区三区演员表 | 精品亚洲永久 | 天天射天天爱天天干 | 亚洲欧洲国产日韩 | 亚洲欧美综合在线一区 | 91精产国品一二三区在线观看 | 日韩人妻无码精品系列专区 | 国产精品偷窥熟女精品视频 | 48沈阳熟女高潮嗷嗷叫 | 深爱五月综合缴情综合网 | 亚洲AV无码成H人动漫无遮挡 | 亚洲九九影院 | 97超碰精品成人国产 | 男人操女人视频免费观看 | 亚欧美无遮挡HD高清在线视频 | 甜性涩爱qvod | 精品粉嫩国产一区二区三区 | 亚洲无人区码一码二码三码四码 | 欧美性受xxxx白人性爽 |