C16orf57 抗体 (AbBy Fluor® 555)
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- 抗原 See all C16orf57 (USB1) 抗体
- C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
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适用
- 人, 小鼠, 大鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This C16orf57 antibody is conjugated to AbBy Fluor® 555
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应用范围
- Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 人, 小鼠, 大鼠
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human C16orf57
- 亚型
- IgG
- 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, Cow, 犬, 豚鼠, 兔, Hamster, 猴, Pig 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
- IF(IHC-P) 1:50-200
- 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
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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
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Synonyms: Chromosome 16 open reading frame 57, CP057_HUMAN, FLJ13154, UPF0406 protein C16orf57.
Background: Involvement in disease,Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). 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.
- 基因ID
- 79650
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