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SPTBN2 Rabbit pAb (bs-10784R)  
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產(chǎn)品編號 bs-10784R
英文名稱 SPTBN2 Rabbit pAb
中文名稱 非紅細(xì)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.  
研究領(lǐng)域 神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Rat (predicted: Mouse,Rabbit,Sheep,Cow,Dog)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 271 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SPTBN2: 71-170/2390 
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(xiàng) 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 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.

SWISS:
O15020

Gene ID:
6712

Database links:

Entrez Gene: 6712 Human

Omim: 604985 Human

SwissProt: O15020 Human

Unigene: 26915 Human



產(chǎn)品圖片
Tissue/cell: human kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat se
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