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Rabbit Anti-Nebulin/PE Conjugated antibody (bs-2401R-PE)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-2401R-PE
英文名称 Rabbit Anti-Nebulin/PE Conjugated antibody
中文名称 PE标记的伴肌动蛋白抗体
别    名 DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  结合蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, )
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nebulin
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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.

具有稳定细肌丝的作用。
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