吃奶呻吟打开双腿做受动态图 -亚洲色偷偷色噜噜狠狠99网-日韩精品极品视频在线观看免费-来一水AV@lysav

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  人才招聘  關(guān)于我們  聯(lián)系我們
蜜桃人妻无码AV天堂三区,日本高清视频www
首頁 > 產(chǎn)品中心 > 一抗 > 產(chǎn)品信息
Rabbit Anti-Dysferlin  antibody (bs-2429R)  
~~~促銷代碼KT202411~~~
訂購熱線:400-901-9800
訂購郵箱:sales@xucheq.com
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@xucheq.com
說明書: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價

產(chǎn)品編號 bs-2429R
英文名稱 Rabbit Anti-Dysferlin  antibody
中文名稱 Dysferlin蛋白抗體
別    名 DMAT; DYSF; Dysferlin (Dystrophy associated fer 1 like protein) (Fer 1 like protein 1); Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive); Dysferlin limb girdle muscular dystrophy 2B; Dystrophy associated fer 1 like 1; Dystrophy associated fer 1 like protein; Dystrophy associated fer1 like 1; Dystrophy associated fer1 like protein; Fer 1 like protein 1; Fer1 like protein 1; FER1L1; FLJ00175; FLJ90168; LGMD 2B; LGMD2B; Limb girdle muscular dystrophy 2B (autosomal recessive) ; Limb girdle muscular dystrophy 2B; Miyoshi myopathy; MM; DYSF_HUMAN.  
研究領(lǐng)域 免疫學(xué)  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Dog,GuineaPig,Horse)
產(chǎn)品應(yīng)用 Flow-Cyt=3ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 237kDa
細(xì)胞定位 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Dysferlin: 1901-2119/2119 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008].

Function:
Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress (By similarity).

Subunit:
Interacts with CACNA1S. Interacts with ANXA1; the interaction is Ca(2+)- and injury state-dependent. Interacts with ANXA2; the interaction is Ca(2+)- and injury state-dependent. Interacts with CACNA1S and PARVB. Interacts with TRIM72/MG53; interaction is required for transport to sites of cell injury during repair patch formation (By similarity). Interacts with CAV3 and PARVB. Interacts with AHNAK; the interaction is direct and Ca(2+)-independent. Interacts with AHNAK2; the interaction is direct and Ca(2+)-independent.

Subcellular Location:
Cell membrane, sarcolemma; Single-pass type II membrane protein. Cytoplasmic vesicle membrane; Single-pass type II membrane protein (By similarity). Note=Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. Detected on the apical plasma membrane of the syncytiotrophoblast. Reaches the plasmma membrane through a caveolin-independent mechanism. Retained by caveolin at the plasmma membrane (By similarity). Colocalizes, during muscle differentiation, with CACNA1S in the T-tubule system of myotubules (By similarity). Accumulates and colocalizes with fusion vesicles at the sarcolemma disruption sites (By similarity).

Tissue Specificity:
Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas.

DISEASE:
Defects in DYSF are the cause of limb-girdle muscular dystrophy type 2B (LGMD2B) . LGMD2B is an autosomal recessive degenerative myopathy characterized by weakness and atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels and slow progression. Scapular muscle involvement is minor and not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs.

Similarity:
Belongs to the ferlin family.
Contains 5 C2 domains.

SWISS:
O75923

Gene ID:
8291

Database links:

Entrez Gene: 508157 Cow

Entrez Gene: 483121 Dog

Entrez Gene: 8291 Human

Entrez Gene: 26903 Mouse

Entrez Gene: 100174519 Orangutan

Entrez Gene: 312492 Rat

Omim: 603009 Human

SwissProt: A6QQP7 Cow

SwissProt: O75923 Human

SwissProt: Q9ESD7 Mouse

Unigene: 252180 Human

Unigene: 220982 Mouse



Dysferlin是與漿膜內(nèi)環(huán)境有關(guān)的一種跨膜蛋白,與肌膜損傷的修復(fù)有關(guān).
產(chǎn)品圖片
Blank control:U937. Primary Antibody (green line): Rabbit Anti-Dysferlin antibody (bs-2429R) Dilution: 2μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody : Goat anti-rabbit IgG-PE Dilution: 1μg /test. Protocol The cells wereincubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
版權(quán)所有 2004-2026 www.xucheq.com 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
欧洲-级毛片内射八十老太婆| 100国产精品人妻无码| 一出一进一爽一粗一大视频| 高清在线播放| 欧美牲交a欧美牲交| 国产偷抇久久精品A片69| 偷窥学校女厕撒尿BBBBB| 肉妇春潮干柴烈火MYFDUCC| 西班牙女郎完整版在线播放| 国产又色又爽无遮挡免费| 灭蟑螂土办法| 日韩人妻无码一区二区三区| 人妻精油按摩BD高清中文字幕| 羞羞漫画在线观看| 久久夜色精品国产噜噜亚洲av| 欧美一区二区三区视频在线观看| 欧美性猛交XXXX乱大交3 | 少妇性按摩无码中文A片| 欧美日韩久久久精品A片| 无码AV中文字幕久久专区| 纯肉大根巨无霸纯黑胡椒火腿肠| 日本熟妇色XXXXX日本免费看| 女人18毛片A级毛片免费视频| 国产九九九九九九九A片| 人妻熟妇乱又伦精品视频| 国产手机精品一区二区| 亚洲成熟丰满熟妇高潮XXXXX| 丰满人妻在公车被猛烈进入电影| 久久久久亚洲AV成人人电影 | 日韩激情| 中字幕视频在线永久在线观看免费 | 将军与娇妻各种做高H| 亚洲AV无码一区二区三区在线 | 女人18毛片a级毛片免费视频| 国产激情无码一区二区三区| 国产亚洲精久久久久久无码| 亚洲亚洲精品AV在线动态图| 欧美成年黄网站色视频| 久久国产加勒比精品无码| 好吊视频一区二区三区| 少妇高潮惨叫久久久久电影69|