PQBP1 抗体 (Middle Region)
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北京 101111
Quick Overview for PQBP1 抗体 (Middle Region) (ABIN1108717)
抗原
See all PQBP1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- Middle Region
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序列
- PSCGLPYYWN ADREEGKERR HHRREELAPY PKSKKAVSRK DEELDPMDPS
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交叉反应 (详细)
- Species reactivity (expected):Mouse, Rat, Bovine, DogSpecies reactivity (tested):Human
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纯化方法
- Purified on Protein A affinity column
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免疫原
- The immunogen for anti-PQBP1 antibody: synthetic peptide directed towards the middle region of human PQBP1.
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亚型
- IgG
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应用备注
- Optimal working dilution should be determined by the investigator.
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限制
- 仅限研究用
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溶解方式
- Add 100 μL of distilled water
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注意事项
- Avoid repeated freezing and thawing.
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储存条件
- -20 °C
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储存方法
- Store the lyophised antibody at -20 °C for up to one year. Store reconstitued antibody undiluted for one month or in aliquots at -20 °C long term.
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有效期
- 12 months
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- PQBP1 (Polyglutamine Binding Protein 1 (PQBP1))
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别名
- PQBP1
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背景
- PQBP1 may suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. It can activate transcription directly or via association with the transcription machinery. PQBP1 may be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit. Defects in PQBP1 are the cause of Renpenning syndrome 1 (RENS1), also known as Sutherland-Haan X-linked mental retardation syndrome (SHS) or X-linked mental retardation syndromes MRXS3/MRXS8/MRX55. The clinical features are mental retardation, microcephaly, short stature, and small testes. The craniofacies tends to be narrow and tall with upslanting palpebral fissures, abnormal nasal configuration, cupped ears, and short philtrum. The nose may appear long or bulbous, with overhanging columella. Less consistent manifestations include ocular colobomas, cardiac malformations, cleft palate, and anal anomalies. RENS1 is more frequently in males than in females where little or no expression is found.Synonyms: 38 kDa nuclear protein containing a WW domain, NPW38, PQBP-1, Polyglutamine tract-binding protein 1, Polyglutamine-binding protein 1
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基因ID
- 10084
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NCBI登录号
- NP_001027553
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途径
- Ribonucleoprotein Complex Subunit Organization
抗原
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