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
亚洲第一网站男人都知道,久久午夜影院,freesex呦交
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-Desmin/Gold Conjugated antibody (bs-20702R-Gold)
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(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-20702R-Gold
英文名稱 Rabbit Anti-Desmin/Gold Conjugated antibody
中文名稱 膠體金標記的結蛋白抗體
別    名 CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  細胞生物  免疫學  信號轉導  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
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 0.4mg/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 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 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。結蛋白是一種中間絲蛋白,廣泛分布于骨骼肌細胞、平滑肌細胞、心肌細胞和肌上皮細胞及其腫瘤中,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AV无码成H人动漫无遮挡 | 欧美婷婷六月丁香综合色 | 国产丝袜在线精品丝袜不卡3D | 伊人国产精品 | 九九99靖品免费 | 青热久思思 | 亚欧日韩欧美网站在线看 | 男人进女人下部全黄大色视频 | 被公牛日到了高潮 | 免费在线a级片 | 69国产精品人妻无码免费 | 少妇人妻呻吟青椒BOBX | 91网站在线观看视频 | 国产最新一区 | 国产群交影片在线播放 | 吃奶呻吟打开双腿做受动态图 | 免费a片在线网站大全 | 91美女在线播放 | 玩偶国产一区二区在线观看 | chinese白浆高潮videos | 久久人人做 | aaaaaaa大片免费看 | 欧美成人一区二区三区 | 98国产精品人妻无码免费 | 囯产丰满肉体A片 | 亚洲欧美另类成人综合图片 | 午夜DJ国产精华日本无码 | 中文字幕乱码亚洲影视 | 17C精品麻豆一区二区免费 | 亚洲码国产精品高潮在线 | 日本在线观看中文自拍 | 人妻偷人VA精品国产旡码 | 一区二区三区网 | 日韩视频在线免费观看 | 久热国产精品视频 | 日本三级无码中文字幕 | 亚洲中文字幕一区二区精品 | 撕开奶罩揉吮奶头视频 | 天堂网91av| 人与禽性视频77777交片 | 在线观看自拍 |