精品国产av自拍_精品日韩欧美在线视频一区二区_一区二区在线观看在线_福利在线观看免费高清完整版_无码免费动漫老黄网站_无码一区在线观看视频_精品五月精品婷婷_免费国产日本高清_亚洲特黄特色一级在线观看_国产Ⅴ亚洲Ⅴ欧美Ⅴ专区

掃碼關(guān)注公眾號(hào)           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
avtt天堂网heyzo系列,久久亚洲一区二区三区,人鲁交YAZHONGHU
Rabbit Anti-Desmin/BF350 Conjugated antibody (bs-1026R-BF350)
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(jià)
產(chǎn)品編號(hào) bs-1026R-BF350
英文名稱 Rabbit Anti-Desmin/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的結(jié)蛋白抗體
別    名 CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  心血管  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞類型標(biāo)志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat,  (predicted: Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, Guinea Pig, )
產(chǎn)品應(yīng)用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 52kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Desmin
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z line structures. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.

Function:
Desmin are class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures.

Subunit:
Homopolymer. Interacts with DST. Interacts with MTM1.

Subcellular Location:
Cytoplasm.

Post-translational modifications:
ADP-ribosylation prevents ability to form intermediate filaments.

DISEASE:
Defects in DES are the cause of myopathy myofibrillar type 1 (MFM1) [MIM:601419]. A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. Note=Mutations in the DES gene are associated with a variable clinical phenotype which encompasses isolated myopathies, pure cardiac phenotypes (including dilated cardiomyopathy, restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy), cardiac conduction disease, and combinations of these disorders. If both cardiologic and neurologic features occur, they can manifest in any order, as cardiologic features can precede, occur simultaneously with, or follow manifestation of generalized neuromuscular disease (PubMed:19879535).
Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.

Similarity:
Belongs to the intermediate filament family.

Database links:

Entrez Gene: 1674 Human

Entrez Gene: 13346 Mouse

Entrez Gene: 64362 Rat

Omim: 125660 Human

SwissProt: P17661 Human

SwissProt: P31001 Mouse

SwissProt: P48675 Rat

Unigene: 594952 Human

Unigene: 6712 Mouse

Unigene: 39196 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Desmin在很多哺乳動(dòng)物中的橫紋肌和各種平滑肌及其來源的腫瘤組織中都有表達(dá)。結(jié)蛋白是一種中間絲蛋白,廣泛分布于骨骼肌細(xì)胞、平滑肌細(xì)胞、心肌細(xì)胞和肌上皮細(xì)胞及其腫瘤中,主要用于子宮、皮膚、胃腸道及其它橫紋肌肉瘤和肌上皮瘤的診斷和鑒別診斷。
版權(quán)所有 2004-2026 0592123.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(hào): 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(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
JAPAN连续高潮喷水VIDEO | 久久久97精品国产一区蜜桃 | 好吊妞视频这里有精品 | 少妇高清精品毛片在线视频 | 在线天堂资源WWW在线污 | 中国黄色一级视频 | 无码AV熟妇素人内射V在线 | 亚洲精品无码av中文字幕网站 | 国产女人高潮叫床男人桶到爽 | yellow片免费观看在线 | 国产精品一区二区三区不卡催乳 | 搡的我好爽视频在线观看 | 国产sanji高清在线视频观看 | 午夜福利麻豆国产精品 | 免费无码片国产在线观看 | 东北熟女高潮内射子宫一插到底 | 国产精品久久久久精品一级APP | 亚洲国产精品免费观看 | 91视频一区二区三区 | 国产黄a一级二级三级看三区 | 成 人影片 免费观看 | 91精品国产高清久久久久久 | baoyu138国产精品tv | j8又粗又硬又大又爽视频 | 欧美人与牲口杂交在线播放免费 | 国产精品一区二区在线播放 | 日韩成人电影在线观看 | 少妇又紧又色又爽又刺激视频 | 综合激情丁香久久狠狠男同 | 国产激情视频在线观看 | 国产成人久久精品二三区麻豆 | 四虎影院免费观看 | 亚洲日本日韩中文字幕狼友版 | 97人妻精品专区久久久久 | 人与禽性视频77777 | 无套内谢少妇毛片AAAA片免费 | 亚洲日产韩国一二三四区 | 无码中文字幕拍偷乱偷精品 | 精品国产免费第一区二区 | 国产真人无码作爱免费看 | 欧美性猛交XXXX乱大交极品 |