TCOF1 抗体 (AA 1-1340)
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Quick Overview for TCOF1 抗体 (AA 1-1340) (ABIN7868120)
抗原
See all TCOF1 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 1-1340
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原理
- TCOF1 Antibody / Treacle protein
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纯化方法
- Immunogen affinity purified
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免疫原
- E.coli-derived human TCOF1 recombinant protein (Position: M1-K1340) was used as the immunogen for the TCOF1 antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the TCOF1 antibody should be determined by the researcher.
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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
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the TCOF1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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- TCOF1 (Treacher Collins-Franceschetti Syndrome 1 (TCOF1))
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别名
- TCOF1
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背景
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TCOF1 antibody detects Treacle protein, a nucleolar phosphoprotein involved in ribosome biogenesis and craniofacial development. The UniProt recommended name is Treacle protein (TCOF1). This protein plays a key role in rRNA transcription, processing, and ribosomal subunit assembly, ensuring proper neural crest cell proliferation during embryogenesis.
Functionally, TCOF1 antibody identifies a 1,488-amino-acid protein localized to the nucleolus, where it interacts with upstream binding factor (UBF) and RNA polymerase I machinery. TCOF1 facilitates 47S pre-rRNA transcription and processing, linking nucleolar activity to cell growth and differentiation. It also participates in DNA damage response by stabilizing nucleolar integrity under stress.
The TCOF1 gene is located on chromosome 5q32-q33.1 and is highly expressed in neural crest-derived and proliferative tissues. Proper TCOF1 function is essential for craniofacial bone and cartilage formation, as neural crest cells depend on efficient ribosome production to sustain rapid proliferation.
Pathologically, haploinsufficiency or mutations in TCOF1 cause Treacher Collins syndrome (TCS), a congenital craniofacial disorder characterized by hypoplasia of facial bones and cleft palate. Defective TCOF1 disrupts ribosome biogenesis, triggering nucleolar stress and apoptosis in neural crest progenitors. Research using TCOF1 antibody supports studies in ribosome assembly, developmental biology, and congenital disease mechanisms.
TCOF1 antibody is validated for western blotting, immunofluorescence, and immunohistochemistry to detect nucleolar proteins involved in ribosome production. NSJ Bioreagents provides TCOF1 antibody reagents optimized for cell biology, developmental genetics, and RNA metabolism research.
Structurally, Treacle protein contains multiple serine-rich and acidic domains that mediate nucleolar localization and protein-protein interactions. This antibody enables analysis of TCOF1's regulatory role in ribosome synthesis and neural crest development. -
UniProt
- Q13428
抗原
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