供货周期: | 一周 |
品牌: | EterLife |
规格: | 0.1ml/100μg 、0.2ml/200μg |
货号: | TE-KT-0183 |
CAS号: | 详见产品说明书 |
脊髓小脑失调症蛋白1抗体
〖别名〗ATX1; ATXN1; SCA1; Ataxin 1; Ataxin-1; Ataxin1; Spinocerebellar ataxia type 1.
〖浓度〗1mg/1ml
〖规格〗0.2ml/200μg
〖抗体来源〗Rabbit
〖克隆类型〗polyclonal
〖交叉反应〗Human, Mouse, Rat
〖产品类型〗脊髓小脑失调症蛋白1抗体一抗
〖研究领域〗染色质和核信号 神经生物学
〖蛋白分子量〗predicted molecular weight: 87kDa
〖性状〗Lyophilized or Liquid
〖免疫原〗KLH conjugated synthetic peptide derived from human Ataxin 1 C-terminus
〖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
〖产品应用〗WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
(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.
〖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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
〖产品介绍〗Ataxin-1, also designated spinocerebellar ataxia type 1 protein (Sca-1), is differentially expressed and localizes to both the cytoplasm and the nucleus. Mutations in Ataxin-1 are associated with the onset of the autosomal dominant neurodegenerative disorder spinocerebellar ataxia type 1 (SCA-1), which is characterized by progressive neuronal loss in the cerebellum, muscle wasting and ataxia. In Purkinje cells, where SCA-1 is predominantly observed, Ataxin-1 has been shown to directly associate with the Purkinje-enriched leucine-rich acidic nuclear protein (LANP) and the nuclear matrix-associated protein promyelocytic leukemia protein PML. In SCA-1, Ataxin-1 is mutated to encode a polyglutamine protein that forms nuclear aggregates, which interact significantly more strongly with LANP and contribute to the pathogenesis of SCA-1.
Function : The exact function of Ataxin 1 is unknown. It has been shown to bind RNA and may be involved in RNA biogenesis. Ataxin 1 is a polyglutamine repeat containing protein whose expansion causes spinocerebellar ataxia type-1 (SCA1)in humans. This also triggers the formation of nuclear inclusions.
Tissue Specificity : Widely expressed throughout the body.
Similarity : Belongs to the ATXN1 family.
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