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
欧美肥妇视频,国产毛片毛片毛片毛片毛片毛片,亚洲一二区视频
Rabbit Anti-Dysferlin/PE Conjugated antibody (bs-2429R-PE)
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-2429R-PE
英文名稱 Rabbit Anti-Dysferlin/PE Conjugated antibody
中文名稱 PE標(biāo)記的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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, )
產(chǎn)品應(yīng)用 Flow-Cyt=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 237kDa
細(xì)胞定位 細(xì)胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Dysferlin
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:
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.

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



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

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).
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: 四川骚妇无套内射舔了更爽 | 穿书自救指南2樱花动漫免费观看 | 久久99热这里只频精品6 | 欧美日韩视频免费 | 无遮挡亲胸捏胸免费视频 | 国产成人无码精品久久久小说 | 免费欧美激情 | 国产精品99久久久久久宅男 | 亚洲AV永久无码精品放毛片 | 国产精品一区三区在线观看 | 国产成年人小视频 | 第九色综合| 性巴克一键去除衣物 | 精品成人一区二区 | 欧美的一卡2卡3卡4卡5在线 | 69久久夜色精品国产7777 | 美女又大又黄www免费网站 | 天地资源在线观看高清 | 免费一级特黄录像 | 91新网址 | 亚洲精品狼友在线播放 | 日本日本乱码伦视频在线观看 | 91麻豆免费在线观看 | 欧美激情视频二区 | 免费无码高潮流白浆视频 | 国产成人综合亚洲欧美 | 香蕉久久国产AV一区二区 | 成人91在线 | 国产精品呻吟声 | 新婚少妇无套内谢国语播放 | 国产98在线 | 久久久久亚洲精品天堂 | 国产色诱视频在线播放网站 | 观看黄色 | 亚洲娇小性XXXXX | 国产精品一品二区三区的使用体验 | 97无码免费人妻超级碰碰碰碰 | 97超碰免费在线观看 | 天天干天天爽 | 91麻豆国产福利在线观看宅福利 | 在线亚洲观看 |