phospho-Ataxin 1 (Ser775) Rabbit pAb, AP conjugated(bs-12534R-AP)-100ul

phospho-Ataxin 1 (Ser775) Rabb

it pAb, AP conjugated(bs-12534R-AP)-100ul
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  • bs-12534R-AP
  • 2025年09月30日
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      100ul

    产品编号bs-12534R-AP
    英文名称phospho-Ataxin 1 (Ser775) Rabbit pAb, AP conjugated
    中文名称AP标记的磷酸化脊髓小脑失调症蛋白1抗体
    英文别名Ataxin 1(phospho Ser776); p-Ataxin 1(phospho S776); ATX1; ATXN1; SCA1; Ataxin 1; Ataxin-1; Ataxin1; Spinocerebellar ataxia type 1; ATX1_HUMAN.
    产品应用WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    抗体来源Rabbit
    免疫原KLH conjugated synthesised phosphopeptide derived from human Ataxin 1 around the phosphorylation site of Ser776
    亚型IgG
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Epigenetics and Nuclear Signaling > DNA / RNA > RNA Processing

    Neuroscience > Neurology process > Neurodegenerative disease

    亚基Homooligomer. Interacts with CIC. Interacts with ANP32A, PQBP1, UBQLN4, ATXN1L, USP7 and ZNF804A. Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1L for RBPJ-binding.
    亚细胞定位Cytoplasm. Nucleus. Note=Colocalizes with USP7 in the nucleus.
    组织特异性Widely expressed throughout the body.
    翻译后修饰Phosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract._x000D_
    Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract.
    相似性Belongs to the ATXN1 family._x000D_
    Contains 1 AXH domain.
    功能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.
    保存条件Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料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).

     

    应用推荐稀释比例
    {WB}{1:500-2000}
    {IHC-P}{1:100-500}
    {IHC-F}{1:100-500}

     

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