產(chǎn)品展示
ATX/Autotaxin/E-NPP2,自分泌運(yùn)動(dòng)因子抗體
點(diǎn)擊次數(shù):123發(fā)布時(shí)間:2015/4/16 9:58:23

更新日期:2016/10/13 9:37:22
所 在 地:美洲
產(chǎn)品型號(hào):
品牌名稱:Abcam
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詳細(xì)內(nèi)容
產(chǎn)品縮寫(xiě) ATXN1
蛋 白 名 Ataxin-1
別 稱 ATX1; SCA1; D6S504E
來(lái) 源 小鼠
特 異 性 抗人
分 子 量 87kDa
克隆類型 單克隆抗體:IgG1;克隆號(hào):2F5
應(yīng) 用 IH(P)免疫組化(石蠟);IC細(xì)胞免疫化學(xué);FC流式細(xì)胞檢測(cè);ELISA
成 分 Ascitic fluid containing 0.03% sodium azide.
免 疫 原 Purified recombinant fragment of human ATXN1 expressed in E. Coli.
保存條件 連續(xù)使用時(shí)4°C存儲(chǔ),保質(zhì)期六個(gè)月;長(zhǎng)期存儲(chǔ)時(shí)建議分裝為10ul以上小包裝-20°C存儲(chǔ),并避免反復(fù)凍融,保質(zhì)期一年。
背景資料 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 contains 41-81 CAG repeats, compared to 6-39 in the normal allele. At least two transcript variants encoding the same protein have been found for this gene.Tissue specificity: Widely expressed throughout the body.
抗體(antibody)指機(jī)體的免疫系統(tǒng)在抗原刺激下,由B淋巴細(xì)胞或記憶細(xì)胞增殖分化成的漿細(xì)胞所產(chǎn)生的、可與相應(yīng)抗原發(fā)生特異性結(jié)合的免疫球蛋白。主要分布在血清中,也分布于組織液及外分泌液中。
抗體的概念:
*初有人用電泳證明血清中抗體活性在γ球蛋白部分,故曾把抗體統(tǒng)稱為丙種(γ)球蛋白。后來(lái)發(fā)現(xiàn),抗體并不都在γ區(qū);并且位于γ區(qū)的球蛋白,也不一定都具有抗體活性。
1964年,世界衛(wèi)生組織舉行專門(mén)會(huì)議,將具有抗體活性以及與抗體相關(guān)的球蛋白統(tǒng)稱為免疫球蛋白(Ig),如骨髓瘤蛋白,巨球蛋白血癥、冷球蛋白血癥等患者血清中存在的異常免疫球蛋白以及正常人天然存在的免疫球蛋白亞單位等。因而免疫球蛋白是結(jié)構(gòu)化學(xué)的概念,而抗體是生物學(xué)功能的概念?梢哉f(shuō),幾乎所有抗體都是免疫球蛋白(極少數(shù)抗體為RNA),但并非所有免疫球蛋白都是抗體。