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
黄色片aaaaaa,色婷婷在线精品国自产拍,好吊妞视频这里有精品
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-FHL1/HRP Conjugated antibody (bs-4827R-HRP)
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-HRP
英文名稱 Rabbit Anti-FHL1/HRP Conjugated antibody
中文名稱 辣根過氧化物酶標記的骨骼肌蛋白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 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=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: 成人亚洲欧美一区二区三区 | 影音先锋中文字幕亚洲资源站 | 国产一区二区三区视频在线 | 中文字幕在线轮第一页 | 国内自拍五区 | 美女又大又黄www免费网站 | 国产超碰人人做人人爽av大片 | 成年视频女人的天堂天天看片 | 成人在线免费观看视频网站 | 国产精品自拍在线观看 | 黄瓜tv在线观看 | 波多野结衣系列18部无码观看a | 好硬好湿好爽再深一点动态图片 | 天天躁日日躁狠狠的躁天龙影院 | 无码中文字幕日韩专区 | 自拍视频一区 | 日本地区不卡一区二区三区搜索 | 人人爽久久涩噜噜噜丁香 | 黄色片一级免费看 | 亚洲国产成人久久综合一区久久久国产99 | 99久久久99久久国产片鸭王 | 国产99久久久国产精品免费看 | 日韩人妻一区二区三区免费 | 中文字幕亚洲综合 | 131美女爽爽爽爱做视频 | 国产精品偷伦视频免费观看了 | 成人影片亚区免费无码 | 国产香蕉97碰碰久久人人九色 | 日本大片免费观看视频 | 中文字幕在线观看视频免费 | 好紧好滑好湿好爽免费视频 | 久久久精品理论A级A片 | 宝贝张开腿嗯啊高潮了视频 | 久久国产免费福利永久 | 国产欧美日韩综合精品二区 | 欧美一区二区一级片 | 午夜资源站 | 国产精品亚洲二区在线观看 | 成熟丰满熟妇高潮XXXXX视频 | 中文字幕国产亚洲 | 成人444kkkk在线观看 |