ATXN7 抗體-ATXN7 Antibody代理/參數/廠家-一抗-艾美捷科技有限公司

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背景資料: The autosomal dominant cerebellar ataxias (ADCA) are anheterogeneous group of neurodegenerative disorders characterized bynprogressive degeneration of the cerebellum, brain stem and spinalncord. Clinically, ADCA has been divided into three groups: ADCAntypes I-III. ADCAI is genetically heterogeneous, with five geneticnloci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6,nbeing assigned to five different chromosomes. ADCAII, which alwaysnpresents with retinal degeneration (SCA7), and ADCAIII oftennreferred to as the 'pure' cerebellar syndrome (SCA5), are mostnlikely homogeneous disorders. Several SCA genes have been clonednand shown to contain CAG repeats in their coding regions. ADCA isncaused by the expansion of the CAG repeats, producing an elongatednpolyglutamine tract in the corresponding protein. The expandednrepeats are variable in size and unstable, usually increasing innsize when transmitted to successive generations. This locus hasnbeen mapped to chromosome 3, and it has been determined that thendiseased allele associated with spinocerebellar ataxia-7 containsn38-130 CAG repeats (near the N-terminus), compared to 7-17 in thennormal allele. The encoded protein is a component of thenSPT3/TAF9/GCN5 acetyltransferase (STAGA) and TBP-freenTAF-containing (TFTC) chromatin remodeling complexes, and it thusnplays a role in transcriptional regulation. Alternative splicingnresults in multiple transcript variants.

免疫原: This ATXN7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 354-381 amino acids from the Central region of human ATXN7.

保存建議: Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

其他: ProSci Incorporated于1998年成立于美國加州圣地亞哥市,從事抗體等科研試劑的研發、生產和銷售。產品涵蓋了細胞凋亡、信號轉導、免疫、腫瘤、細胞生物、神經生物、傳染病等領域。ProSci擁有超過30000種單克隆抗體和多克隆抗體。ProSci的抗體大都經免疫親和層析純化,具有高純度、高親和力、特異性強等特點,所有的ProSci抗體都經過了嚴格的驗證測試和質量控制,已保證高質量的產品。此外,ProSci還提供標記二抗,重組蛋白,細胞和組織裂解液,細胞涂片,組織切片,多肽等產品與抗體配套使用。