Ataxin 3 抗体 (C-Term) (APC)
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Quick Overview for Ataxin 3 抗体 (C-Term) (APC) (ABIN7970575)
抗原
See all Ataxin 3 (ATXN3) 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- C-Term
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原理
- Anti-Ataxin 3/ATXN3 Antibody APC Conjugated
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特异性
- No cross reactivity with other proteins.
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交叉反应 (详细)
- No cross-reactivity with other proteins
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预测反应
- different from the related mouse,rat sequences by two amino acids.
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纯化方法
- Immunogen affinity purified.
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免疫原
- A synthetic peptide corresponding to a sequence at the C-terminus of human Ataxin 3, different from the related mouse and rat sequences by two amino acids.
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亚型
- IgG
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应用备注
- Flow Cytometry, Optimal dilutions should be determined by end users.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % Sodium azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
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有效期
- 12 months
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- Ataxin 3 (ATXN3)
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别名
- ATXN3
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背景
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Background: ATXN3 (Ataxin 3), also known as AT3, MJD GENE, MJD1, SCA3 GENE, ATX3, JOS, Spinocerebellar ataxia-3, Machado-Joseph disease protein 1, is a protein that in humans is encoded by the ATXN3 gene. ATXN3 ranges in size from 360 to 374 amino acids. Using Northern blot analysis showed that ATXN3 mRNA was ubiquitously expressed in human tissues. They detected at least 4 ATXN3 transcripts of 1.4, 1.8, 4.5, and 7.5 kb and suggested that the different mRNA species probably result from differential splicing and polyadenylation. Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by the ATXN3 gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is an inverse correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. Ataxin-3 interacted with 2 human homologs of the yeast DNA repair protein RAD23, HHR23A (RAD23A) and HHR23B (RAD23B). Both normal and mutant ataxin-3 proteins interacted with the ubiquitin-like domain at the N terminus of the HHR23 proteins, which is a motif important for nucleotide excision repair. However, in HEK 293 cells, HHR23A was recruited to intranuclear inclusions formed by the mutant ataxin-3 through its interaction with ataxin-3.
Gene Full Name: ataxin 3
Sequence Similarities: Contains 1 Josephin domain.
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基因ID
- 4287
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UniProt
- P54252
抗原
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