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
成人午夜福利视频,国产精品无码AV天天爽播放器,永夜星河在线免费观看
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-Dysferlin/FITC Conjugated antibody (bs-2429R-FITC)
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  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2429R-FITC
英文名稱 Rabbit Anti-Dysferlin/FITC Conjugated antibody
中文名稱 FITC標記的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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 免疫學  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, )
產品應用 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
細胞定位 細胞膜 
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是與漿膜內環境有關的一種跨膜蛋白,與肌膜損傷的修復有關.
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: 男生操女生视频免费看 | 成人深夜在线 | 精品二区在线 | 在线观看qvod| 日韩高清免费在线 | 免费看av在线网站网址 | 美女不带套日出白浆免费视频 | 成人美女黄网站色大免费的 | 亚洲国产xxxx | 国内精品视频在线观看九九 | 成人久久视频 | 欧美日一 | 无码成人一区二区 | 91精品一区二区三区蜜臀 | 久久成人人人人精品欧 | 免费中午字幕无吗 | 制服丝袜国产在线无码 | 国产成人亚洲精品影院 | 伊人久久情人综岁的合网 | 城中村勾搭老熟女啪啪 | 欧美伊人精品成人久久综合97 | 国产一区二区三区久久20p | 亚洲精品乱码久久久久久蜜桃91 | 在线观看亚洲AV日韩A∨ | 日本熟妇牲交视频 | 超清无码无卡中文字幕 | 一本久久a久久精品合男女 韩国成人免费视频 | 国产成人啪精品午夜网站 | 人妻AV无码一区二区三区 | 亚洲美女一级毛片 | 亚洲va无码va在线va天堂 | 国产精品一区二区久久精品爱涩 | 337P日本欧洲亚洲大胆 | 日日干夜夜操天天操 | 久草成人在线 | 欧美xx视频 | a级免费毛片 | 99久久99久久免费精品小说 | 日本无码潮喷A片无码高潮 欧美变态另类人妖 | 亚洲一本之道高清乱码 | 国产精品v欧美精品v日韩精品v |