產(chǎn)品概述
| 產(chǎn)品名稱（Product Name） | Ataxin-1 Rabbit Polyclonal Antibody |
| 描述（Description） | Rabbit Polyclonal Antibody |
| 宿主（Host） | Rabbit |
| 應(yīng)用（Application） | WB,IHC-P,IF-P,IF-F,ICC/IF,ELISA |
| 種屬反應(yīng)性（Reactivity） | Human,Mouse |
產(chǎn)品性能
| 偶聯(lián)物（Conjugation） | Unconjugated |
| 修飾（Modification） | Unmodified |
| 同種型（Isotype） | IgG |
| 克?。–lonality） | Polyclonal |
| 形式（Form） | Liquid |
| 存放說明（Storage） | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 儲(chǔ)存溶液（Buffer） | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N. |
| 純化方式（Purification） | Affinity purification |
免疫原
| 基因名（Gene Name） | ATXN1 |
| 別名（Alternative Names） | ATXN1; ATX1; SCA1; Ataxin-1; Spinocerebellar ataxia type 1 protein |
| 基因ID（Gene ID） | 6310 |
| 蛋白ID（SwissProt ID） | P54253 |
產(chǎn)品應(yīng)用
| 稀釋比（Dilution Ratio） | WB 1:500-2000, IHC-P 1:100-1:300, IF-P/IF-F/ICC/IF 1:200-1:1000, ELISA 1:5000.Not yet tested in other applications. |
| 蛋白分子量（Molecular Weight） | 87kDa |
研究背景
ataxin 1(ATXN1) Homo sapiens 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 transmittedalternative products:At least 2 isoforms are produced,disease:Defects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1) [MIM:164400]; also known as olivopontocerebellar atrophy I (OPCA I or OPCA1). 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 cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. SCA1 is caused by expansion of a CAG repeat in the coding region of ATXN1. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.,domain:The AXH domain is required for interaction with CIC.,function:Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.,miscellaneous:The self-association seems to be necessary to form nuclear aggregates.,online information:Ataxin-1 entry,polymorphism:The poly-Gln region of ATXN1 is highly polymorphic (4 to 39 repeats) in the normal population and is expanded to about 40-83 repeats in spinocerebellar ataxia 1 (SCA1) patients.,similarity:Belongs to the ATXN1 family.,similarity:Contains 1 AXH domain.,subcellular location:Colocalizes with USP7 in the nucleus.,subunit:Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBIN, ATXN1L, USP7 and ZNF804A.,tissue specificity:Widely expressed throughout the body.,
研究領(lǐng)域
關(guān)鍵字： ATXN1;Ataxin-1;Rabbit;Polyclonal;Antibody;一抗
武漢恩璣生命科技有限公司（EnkiLife）是一家深耕生命科學(xué)，專注細(xì)胞生物學(xué)和免疫學(xué)科研試劑的研發(fā)、生產(chǎn)與銷售的生物技術(shù)企業(yè)，在全球100多個(gè)國家和地區(qū)開展業(yè)務(wù)，致力于為科研工作者提供高質(zhì)量的產(chǎn)品和卓越的客戶服務(wù)，推動(dòng)生命科學(xué)的發(fā)展。
EnkiLife的產(chǎn)品線涵蓋細(xì)胞系、原代細(xì)胞、細(xì)胞培養(yǎng)基、血清、細(xì)胞檢測(cè)試劑盒、重組靶點(diǎn)蛋白、細(xì)胞因子、重組抗體、ELISA試劑盒、生化試劑盒等，并提供技術(shù)服務(wù)與定制開發(fā)，覆蓋了生命科學(xué)研究的各大關(guān)鍵領(lǐng)域，包括細(xì)胞生物學(xué)、癌癥、免疫學(xué)、神經(jīng)科學(xué)、心血管疾病、干細(xì)胞、表觀遺傳學(xué)、內(nèi)分泌、蛋白質(zhì)組學(xué)、代謝組學(xué)等，全方位滿足您的實(shí)驗(yàn)需求，讓您享受科研的樂趣！
公司現(xiàn)已建立四大技術(shù)平臺(tái)：
EnCyto?細(xì)胞培養(yǎng)及檢測(cè)平臺(tái)：擁有細(xì)胞系庫（500+）、原代細(xì)胞庫（500+）、基礎(chǔ)培養(yǎng)基和完全培養(yǎng)基（1200+）
EnkiPro?重組蛋白平臺(tái)：現(xiàn)貨產(chǎn)品2000+，可提供定制化表達(dá)服務(wù)
EnAb?重組抗體平臺(tái)：重組兔單抗（3000+），可提供定制化和標(biāo)記服務(wù)
EnKits?試劑盒開發(fā)平臺(tái)：可提供優(yōu)質(zhì)的ELISA試劑盒、配套試劑、抗體對(duì)、生化試劑盒等相關(guān)產(chǎn)品
EnkiLife在生產(chǎn)管理方面引入ISO9001質(zhì)量管理體系和信息化、自動(dòng)化的管理工具，擁有高效穩(wěn)定的交付能力，與全球知名品牌建立了緊密的合作。
EnkiLife始終堅(jiān)持以技術(shù)創(chuàng)新為驅(qū)動(dòng)，以匠心鑄就品質(zhì)，以品質(zhì)服務(wù)客戶。
我們期待與更多的全球科研工作者和企業(yè)攜手合作，共同推動(dòng)生命科學(xué)領(lǐng)域的進(jìn)步與發(fā)展。