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Rabbit Anti-BEAN1/HRP Conjugated antibody (bs-11710R-HRP)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-11710R-HRP
英文名称 Anti-BEAN1/HRP
中文名称 辣根过氧化物酶标记的脊髓小脑共济失调蛋白BEAN1抗体
别    名 BEAN; SCA31; Bean1; BEAN1_HUMAN; Brain-expressed protein associating with Nedd4 homolog; Protein BEAN1; SCA31.  
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  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, )
产品应用 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 29kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BEAN1 (10-70aa)
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:
The protein encoded by this gene is one of several proteins that interact with NEDD4, a member of a family of ubiquitin-protein ligases. These proteins have PY motifs in common that bind to the WW domains of NEDD4. NEDD4 is developmentally regulated, and is highly expressed in embryonic tissues. Mutations in this gene (i.e., intronic insertions of >100 copies of pentanucleotide repeats including a (TGGAA)n sequence) are associated with spinocerebellar ataxia type 31. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2010].

Subunit:
Interacts with NEDD4

Subcellular Location:
Membrane; Single-pass membrane protein (Potential).

DISEASE:
Defects in BEAN1 are the cause of spinocerebellar ataxia type 31 (SCA31) [MIM:117210]; also known as spinocerebellar ataxia 16q22-linked. A form of spinocerebellar ataxia, 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. SCA31 belongs to the autosomal dominant cerebellar ataxias type III (ADCA III) which are characterized by pure cerebellar ataxia without additional signs.

Database links:

Entrez Gene: 146227 Human

Omim: 612051 Human

SwissProt: Q3B7T3 Human

Unigene: 97805 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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