Ataxin 3 抗体 (C-Term)
Quick Overview for Ataxin 3 抗体 (C-Term) (ABIN3043796)
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
- AA 226-254, C-Term
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原理
- Anti-Ataxin 3/ATXN3 Antibody Picoband®
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序列
- EEDLQRALAL SRQEIDMEDE EADLRRAIQ
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交叉反应 (详细)
- No cross-reactivity with other proteins
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产品特性
- Anti-Ataxin 3/ATXN3 Antibody Picoband® (ABIN3043796). Tested in Flow Cytometry, IF, IHC, ICC, WB applications. This antibody reacts with Human, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
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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
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Western blot, 0.1-0.5 μg/mL, Human, Rat
Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human1. Burnett, B., Li, F., Pittman, R. N. The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum. Molec. Genet. 12: 3195-3205, 2003. 2. Paulson, H. L., Das, S. S., Crino, P. B., Perez, M. K., Patel, S. C., Gotsdiner, D., Fischbeck, K. H., Pittman, R. N.Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.Ann. Neurol. 41: 453-462, 1997. -
说明
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Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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浓度
- 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.
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注意事项
- Avoid repeated freezing and thawing.
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储存条件
- 4 °C,-20 °C
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储存方法
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Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
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- Ataxin 3 (ATXN3)
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别名
- ATXN3
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背景
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Synonyms: Ataxin-3,3.4.19.12,Machado-Joseph disease protein 1,Spinocerebellar ataxia type 3 protein,ATXN3,ATX3, MJD, MJD1, SCA3,
Tissue Specificity: Ubiquitous.
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.
Sequence Similarities: Contains 1 Josephin domain.
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分子量
- 42 kDa
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基因ID
- 4287
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UniProt
- P54252
抗原
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