| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 1/50-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | FIB; FLRN; Nop1; RNU3IP1 |
| Entrez GeneID | 2091 |
| WB Predicted band size | Calculated MW: 34 kDa; Observed MW: 34 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse,Rat |
| Immunogen | A synthesized peptide derived from human Fibrillarin |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
+ +
以下是关于Fibrillarin抗体的3篇参考文献概览：
---
1. **文献名称**: *Autoantibodies to fibrillarin in systemic sclerosis*
**作者**: Reichlin, M., et al.
**摘要**: 该研究分析了系统性硬化症(硬皮病)患者中抗Fibrillarin抗体的临床意义，发现此类抗体与弥漫性皮肤病变和内脏器官受累(如肺纤维化)显著相关，提示其作为疾病亚型的潜在生物标志物。
---
2. **文献名称**: *Fibrillarin autoantibodies in systemic sclerosis: frequency and clinical correlation*
**作者**: Okano, Y., et al.
**摘要**: 通过ELISA和免疫印迹法检测发现，抗Fibrillarin抗体在系统性硬化症患者中特异性较高，且与年轻患者和快速进展的肾脏并发症相关，强调其在早期诊断中的价值。
---
3. **文献名称**: *The role of fibrillarin in ribosome biogenesis and cellular stress response*
**作者**: Tollervey, D., et al.
**摘要**: 该基础研究阐明了Fibrillarin蛋白在核仁rRNA加工中的关键作用，并发现其在细胞应激(如DNA损伤)时发生修饰，可能解释自身抗体在特定病理条件下的产生机制。
---
4. **文献名称**: *Anti-fibrillarin antibodies in patients with overlap syndromes*
**作者**: Satoh, M., et al.
**摘要**: 研究发现重叠综合征(如系统性硬化-狼疮混合)患者中抗Fibrillarin抗体的存在与独特临床表型相关，提示其可能反映不同自身免疫疾病间的分子交叉反应。
---
以上文献涵盖临床相关性、诊断价值及分子机制研究，时间跨度从基础科学到临床应用。
Fibrillarin antibodies are autoantibodies targeting fibrillarin, a highly conserved nucleolar protein involved in ribosomal RNA (rRNA) processing and ribosome biogenesis. Fibrillarin, a component of small nucleolar ribonucleoprotein (snoRNP) complexes, plays a critical role in site-specific methylation of pre-rRNA and nucleolar structure organization. These antibodies are primarily associated with systemic autoimmune diseases, particularly systemic sclerosis (scleroderma), where they serve as a diagnostic marker.
First identified in the 1980s, fibrillarin antibodies are detected in approximately 5-10% of systemic sclerosis patients, often correlating with diffuse cutaneous involvement, severe organ manifestations (e.g., pulmonary hypertension), and poorer prognosis. They are also linked to overlap syndromes, such as scleroderma-polymyositis, and occasionally observed in systemic lupus erythematosus (SLE) or hepatitis C-related autoimmune conditions.
In clinical diagnostics, fibrillarin antibodies are identified via indirect immunofluorescence (IIF), showing a characteristic clumpy nucleolar staining pattern on HEp-2 cells, and confirmed by immunoprecipitation or ELISA. Beyond diagnostics, these antibodies are research tools for studying nucleolar function, RNA metabolism, and autoimmune pathogenesis. Their presence highlights dysregulation of immune tolerance to nucleolar components, offering insights into disease mechanisms and potential therapeutic targets.
×
