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
成人无码a级毛片免费,欧美午夜a级限制福利片,久草久在线
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-Dysferlin/AP Conjugated antibody (bs-2429R-AP)
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-AP
英文名稱 Rabbit Anti-Dysferlin/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)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
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 237kDa
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: 四虎永久免费地址入口 | 青青青视频香蕉在线观看视频 | 精品国产人免费观看成人片 | 亚洲视频一| 亚洲香蕉视频综合在线 | 一级做a爰大片免费 | 成年人视频在线免费 | 色综合久久无码中文字幕 | 熟妇人妻无码中文字幕老熟妇 | 久久午夜无码免费 | 亚洲在线不卡 | 一本色道精品久久一区二区三区 | 久久精品aaaaaa羞羞羞 | 欧美激情综合亚洲一二区 | 国产精品一区二区精品视频免费看 | 青青草视频观看 | 国产妇女馒头高清泬20P多毛 | 久久国产午夜精品理论片34页 | 男人插女人骚视频 | 成人影院在线免费观看 | 奇米国产在线 | 国产成人亚洲综合a∨婷婷图片 | 香蕉成人国产精品免费看网站 | 蝌蚪成人网 | 色综合天天综合高清 | 国产成人综合在线女婷五月99播放 | 西西午夜影院 | 国产精品久久9 | 亚欧日韩欧美网站在线看 | 久久精品爱爱视频 | 国产午夜亚洲精品不卡在线观看 | 久久狠狠爱| 免费观看好看的国产片 | 国产日产欧洲无码视频无遮挡 | 欧美一区日本一区韩国一区 | 成人乱淫av日日摸夜夜爽 | 日韩高清影片在线观看 | 学生妹av| 日韩一区二区三区视频在线观看 | 九七视频在线观看 | 久久午夜无码鲁丝片 |