磷酸化失调症蛋白1抗体

英文名称    phospho-ATXN1(Thr236)    

中文名称    磷酸化失调症蛋白1抗体    

别    名    ATXN1; ATX1; D6S504E; SCA1; Ataxin-1; Spinocerebellar ataxia type 1; ATX1_RAT.    

说 明 书    0      

产品类型    磷酸化抗体     

研究领域    肿瘤  免疫学  神经生物学  信号转导  细胞凋亡  转录调节因子      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Mouse, Rat,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500磷酸化失调症蛋白1抗体 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    90kDa    

细胞定位    细胞核 细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated Synthesised phosphopeptide derived from human ATXN1 around the phosphorylation site of Thr236    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

磷酸化失调症蛋白1抗体产品介绍    background:

The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the `pure' cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains41-81 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1).

Function:
Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.    

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