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Nebulin Rabbit pAb (bs-2401R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價
產(chǎn)品編號 bs-2401R
英文名稱 Nebulin Rabbit pAb
中文名稱 伴肌動蛋白抗體
別    名 DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505.  
研究領(lǐng)域 細胞生物  免疫學(xué)  結(jié)合蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應(yīng) (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Chicken,Dog)
產(chǎn)品應(yīng)用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 500-800 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nebulin: 6401-6669/6669 
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 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.

SWISS:
P20929

Gene ID:
4703

Database links:

Entrez Gene: 4703 Human

Entrez Gene: 17996 Mouse

Entrez Gene: 311029 Rat

Omim: 161650 Human

SwissProt: P20929 Human

Unigene: 588655 Human



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