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Anti-Ataxin 7脊髓小脑共济失调蛋白7抗体
点击次数：189发布时间：2012/12/26 1:44:53
更新日期：2024/9/5 14:43:27
所 在 地：其它
产品型号：BY-11318R
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货号：BY-11318R
英文名称：Anti-Ataxin 7
中文名称：脊髓小脑共济失调蛋白7抗体
其他名称：Ataxin7;Ataxin-7;ADCAII;ATXN 7;OPCA III;OPCA3;SCA 7;SCA7;Spinocerebellar Ataxia 7;Spinocerebellar ataxia type 7 protein.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 96kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat
产品介绍：The human ataxin-7 gene, also known as spinocerebellar ataxia 7 or SCA7, maps to chromosome 3p13-p12, has a 2,727-bp open reading frame, ａnd encodes a 892 amino acid protein containing a nuclear localization signal ａnd a polyglutamine tract （1,2）. SCA7 is an autosomal dominant neurodegenerative disorder characterized by ataxia ａnd selective neuronal cell loss caused by the expansion of a translated CAG repeat encoding a polyglutamine tract in ataxin-7, which is the SCA7 gene product （3,4）. Ataxin-7 is expressed within neurons both affected ａnd unaffected in SCA7 pathology with subcellular localization being variable depending upon the neuronal subtype （5）. Polyglutamine expanded in ataxin-7 may carry out its pathogenic effects in the nucleus by altering the matrix-associated nuclear structure and/or by disrupting nucleolar function （6）.Function : Ataxin 7 is a protein of unknown function. It may be the human orthologue of the yeast SAGA SGF73 subunit ａnd a subunit of the human TFTC-like transcriptional complexes. Spinocerebellar ataxia 7 （one of a group of hereditary neurodegenrative diseases） is caused by an expanded trinucleotide repeat in the gene encoding ataxin 7. Ataxin 7 is typically located in the cytoplasm ａnd on the nuclear membrane of normal brain neurons. In cells where there is a mutation of the SCA7 gene, ataxin 7 accumulates in intranuclear inclusions ａnd can result in cell death. Subcellular Location : Cytoplasmic （isoform b） ａnd Nuclear （isoform a）
