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
国产女人水真多18毛片18精品,99草视频在线观看,韩日av一区二区
Rabbit Anti-FHL1/Cy5.5 Conjugated antibody (bs-4827R-Cy5.5)
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-4827R-Cy5.5
英文名稱 Rabbit Anti-FHL1/Cy5.5 Conjugated antibody
中文名稱 Cy5.5標(biāo)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(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  轉(zhuǎn)錄調(diào)節(jié)因子  鋅指蛋白  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Sheep, )
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 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(chǎn)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(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: 中文字幕av免费观看 | 538国产精品一区二区在线 | 一久久久 | 国产精品毛片一区视频播不卡 | 国产性xxxx高清 | 久久艹伊人 | 视频在线看 | 久久久久久久久女人体 | 亚洲av片不卡无码影视 | 98国产精品人妻无码免费 | 久久嫩草视频 | 91夜色| 国产二区精品视频 | 国产乱码久久久久久 | 中日韩中文字幕区 | a级片在线免费观看视频 | 亚洲伊人久久精品酒店 | 四虎影视在线看免费观看 | 中文字幕第35页 | 老司机亚洲精品一区二区 | av最新在线观看 | 男女做爰裸体猛烈吃奶摸视频 | 久久二区视频 | 色香欲综合成人免费视频 | 高潮流白浆潮喷在线播放视频 | 91视频影院 | 国产一级片久久 | 日日草av | 国产精品Va免费视频 | 国产精品入口福利 | 国产成人午夜片在线观看 | 亚洲国产精品一区久久av篠田 | 美女内射毛片在线看 | 三区av| 无码国产精品一区二区免费虚拟VR | 婷婷久久久亚洲欧洲日产国码AV | 夜夜躁狠狠躁日日躁202 | 一区二区三区在线观看欧美 | 欧美S码亚洲码精品M码 | 中文字幕无码日韩中文字幕 | 91免费看. |