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Rabbit Anti-Nebulin/BF555 Conjugated antibody (bs-2401R-BF555)
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訂購郵箱:sales@73327.net
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2401R-BF555
英文名稱1 Rabbit Anti-Nebulin/BF555 Conjugated antibody
中文名稱 BF555標記的伴肌動蛋白抗體
別    名 DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  結合蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 500-800kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nebulin
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:
Nebulin is a high molecular weight protein (500-800 kD), localized at the N2-line of skeletal muscle myofibrils. It exhibits an elastic stretch dependence, as it maintains the same proportional distance between the z-line and the m-line as the sarcomere is stretched.

Function:
This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Bind and stabilize F-actin.

Subcellular Location:
Cytoplasm > myofibril > sarcomere. Cytoplasm > cytoskeleton.

Tissue Specificity:
Muscle specific. Located in the thin filament of striated muscle.

DISEASE:
Defects in NEB are the cause of nemaline myopathy type 2 (NEM2) [MIM:256030]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination.

Similarity:
Contains 178 nebulin repeats.
Contains 1 SH3 domain.

Database links:

Entrez Gene: 4703 Human

Entrez Gene: 17996 Mouse

Entrez Gene: 311029 Rat

Omim: 161650 Human

SwissProt: P20929 Human

Unigene: 588655 Human



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

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