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- 抗原 See all C16orf57 (USB1) 抗体
- C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
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抗原表位
- AA 154-167, Internal Region
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适用
- 人, Pig
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宿主
- 山羊
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克隆类型
- 多克隆
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标记
- This C16orf57 antibody is un-conjugated
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应用范围
- Western Blotting (WB), Enzyme Immunoassay (EIA)
- 序列
- C-TANQVKIYTN QEKT
- 特异性
- This antibody is expected to recognize both reported isoforms (NP_078874.2, NP_001182231.1).
- 交叉反应 （详细）
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Species reactivity (expected):Pig.
Species reactivity (tested):Human, Mouse, Rat. - 纯化方法
- Ammonium Sulphate Precipitation followed by Antigen Affinity Chromatography using the immunizing peptide
- 免疫原
- Peptide with sequence from the internal region of the protein sequence according to NP_078874.2, NP_001182231.1.
- Top Product
- Discover our top product USB1 Primary Antibody
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anti-U6 SnRNA Biogenesis 1 (USB1) (AA 1-90) antibody
USB1 适用: 人 ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-U6 SnRNA Biogenesis 1 (USB1) (AA 154-167) antibodyVerified USB1 适用: 人, 小鼠, 大鼠 WB, ELISA 宿主: 山羊 Polyclonal unconjugated
anti-U6 SnRNA Biogenesis 1 (USB1) (AA 1-265) antibodyUSB1 适用: 人 WB 宿主: 小鼠 Polyclonal unconjugated
anti-U6 SnRNA Biogenesis 1 (USB1) (AA 150-199) antibodyUSB1 适用: 人, 小鼠, 大鼠, 马, Pig, Cow, 兔, 犬, 豚鼠, 猴, Hamster WB 宿主: 兔 Polyclonal unconjugated
anti-U6 SnRNA Biogenesis 1 (USB1) (AA 1-265) antibodyUSB1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
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- 应用备注
- Optimal working dilution should be determined by the investigator.
- 限制
- 仅限研究用
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- 浓度
- 0.5 mg/mL
- 缓冲液
- Tris saline, pH ~7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer
- 储存液
- Sodium azide
- 注意事项
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- 注意事项
- Avoid repeated freezing and thawing.
- 储存条件
- 4 °C/-20 °C
- 储存方法
- Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
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- 抗原
- C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
- 别名
- C16orf57 (USB1 产品)
- 别名
- C16orf57 antibody, HVSL1 antibody, Mpn1 antibody, PN antibody, hUsb1 antibody, C18H16orf57 antibody, AA960436 antibody, RGD1305215 antibody, c16orf57 antibody, C2H16orf57 antibody, zgc:91896 antibody, U6 snRNA biogenesis phosphodiesterase 1 antibody, U6 snRNA biogenesis 1 antibody, U6 snRNA biogenesis phosphodiesterase 1 L homeolog antibody, USB1 antibody, Usb1 antibody, usb1.L antibody, usb1 antibody
- 背景
- Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN) [MIM:604173]. PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.Synonyms: FLJ13154, UPF0406 protein C16orf57
- 基因ID
- 79650
- NCBI登录号
- NP_078874
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