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货号： bs-20343R-AF594 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-20343R-AF594
- 英文名称
- Anti-SPTBN2/AF594
- 中文名称
- AF594标记的非红细胞血影脑蛋白β2/spectrin β III抗体
- 别 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 神经生物学 信号转导 细胞骨架
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Horse,
- 产品应用
- 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 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human SPTBN2
- 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:
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.