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Rabbit Anti-SPTBN2/BF594 Conjugated antibody (bs-10784R-BF594)
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-10784R-BF594
英文名稱 Rabbit Anti-SPTBN2/BF594 Conjugated antibody
中文名稱 BF594標記的非紅細胞血影腦蛋白β2/spectrin β III抗體
別    名 Beta III spectrin; Beta-III spectrin; SCA 5; SCA5; Spectrin beta chain brain 2; Spectrin beta non erythrocytic 2; Spectrin non erythroid beta chain 2; Spinocerebellar ataxia 5; SPTBN 2; Spectrin beta chain, non-erythrocytic 2; Beta-III spectrin; Spinocerebellar ataxia 5 protein; SPTN2_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 神經生物學  信號轉導  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Rat,  (predicted: Mouse, Dog, Cow, Rabbit, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 271kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SPTBN2
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:
Spectrin is an actin binding protein that is a major component of the plasma membrane skeleton. Spectrins function as membrane organizers and stabilizers by forming dimers, tetramers and higher polymers. Spectrin Alpha and spectrin Beta are present in erythrocytes, whereas spectrin Alpha II (also designated fodrin Alpha) and spectrin Beta I (also designated fodrin Beta) are present in other somatic cells. The spectrin tetramers in erythrocytes act as barriers to lateral diffusion, but spectrin dimers seem to lack this function. Spectrin Beta III is highly homologous to both spectrin Beta I and spectrin Beta II. Western blot analysis shows that spectrin Beta III migrates at a higher molecular mass than predicted in the kidney. Spectrin Beta III is highly expressed in brain, kidney, pancreas, and liver, and at lower levels in lung and placenta. Specifically, spectrin Beta III constitutes a major component of the Golgi and vesicular membrane skeletons.

Function:
Probably plays an important role in neuronal membrane skeleton.

Subcellular Location:
Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.

Tissue Specificity:
Highly expressed in brain, kidney, pancreas, and liver, and at lower levels in lung and placenta.

DISEASE:
Defects in SPTBN2 are the cause of spinocerebellar ataxia type 5 (SCA5) [MIM:600224]. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA5 is an autosomal dominant cerebellar ataxia (ADCA). It is a slowly progressive disorder with variable age at onset, ranging between 10 and 50 years.

Similarity:
Belongs to the spectrin family.
Contains 2 CH (calponin-homology) domains.
Contains 1 PH domain.
Contains 17 spectrin repeats.

Database links:

Entrez Gene: 6712 Human

Omim: 604985 Human

SwissProt: O15020 Human

Unigene: 26915 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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