原发性硬化性胆管炎 (PSC) 是一种影响胆管的持续性炎症性肝脏疾病，通常在年轻人中诊断出来。尽管努力整合各种临床、生化和分子参数来诊断 PSC，但它仍然具有挑战性，并且迄今为止尚未鉴定出该疾病的生物标志物特征。 PSC 与不确定的预后相关，迫切需要探索多组学数据库来建立新的生物标志物组，以早期检测 PSC 逐渐进展为胆管癌 (CCA) 并开发有效的治疗干预措施。除了非编码 RNA 之外，核糖核蛋白 (RNP) 复合物的其他成分，例如 RNA 结合蛋白 (RBP)，由于其在病理条件下的多功能表达，也有望作为生物标志物。在本综述中，提供了 RBP 转录本的更新，显示 PSC 和 CCA 中表达失调。此外，通过利用生物信息学数据挖掘方法，我们深入了解了那些在肝脏、胆囊以及体液中也表现出差异表达的 RBP 转录本，并且有望作为诊断和预测 PSC 预后的生物标志物。使用 CCA 的 TCGA 胆管癌数据集和 PSC 和 CCA 的特定 NCBI GEO 数据集从公共存储库中以生物信息方式提取表达数据；更具体地说，RBPs注释是从RBP World数据库获得的。有趣的是，我们的综合分析显示，与各自的对照组相比，PSC 和 CCA 患者体液中非典型 RBP、FANCD2 以及微管动力学调节因子 ASPM 转录物的表达升高，与对照组的趋势相同。在胆囊和肝癌组织中观察到表达。因此，操纵 RBP 转录本的组织表达可能被认为是减轻 PSC 患者 CCA 发作的一种策略，并且值得进一步的实验研究。本文进行的分析可能有助于鉴定非侵入性生物标志物，用于早期检测 PSC 并预测其进展为 CCA。总之，未来的临床研究应该更深入地研究 RBP 转录本作为人类病理学生物标志物的全部潜力。版权所有 © 2024 Ala 和 Fagoonee。

Primary Sclerosing Cholangitis (PSC) is a persistent inflammatory liver condition that affects the bile ducts and is commonly diagnosed in young individuals. Despite efforts to incorporate various clinical, biochemical and molecular parameters for diagnosing PSC, it remains challenging, and no biomarkers characteristic of the disease have been identified hitherto. PSC is linked with an uncertain prognosis, and there is a pressing need to explore multiomics databases to establish a new biomarker panel for the early detection of PSC's gradual progression into Cholangiocarcinoma (CCA) and for the development of effective therapeutic interventions. Apart from non-coding RNAs, other components of the Ribonucleoprotein (RNP) complex, such as RNA-Binding Proteins (RBPs), also hold great promise as biomarkers due to their versatile expression in pathological conditions. In the present review, an update on the RBP transcripts that show dysregulated expression in PSC and CCA is provided. Moreover, by utilizing a bioinformatic data mining approach, we give insight into those RBP transcripts that also exhibit differential expression in liver and gall bladder, as well as in body fluids, and are promising as biomarkers for diagnosing and predicting the prognosis of PSC. Expression data were bioinformatically extracted from public repositories usingTCGA Bile Duct Cancer dataset for CCA and specific NCBI GEO datasets for both PSC and CCA; more specifically, RBPs annotations were obtained from RBP World database. Interestingly, our comprehensive analysis shows an elevated expression of the non-canonical RBPs, FANCD2, as well as the microtubule dynamics regulator, ASPM, transcripts in the body fluids of patients with PSC and CCA compared with their respective controls, with the same trend in expression being observed in gall bladder and liver cancer tissues. Consequently, the manipulation of tissue expression of RBP transcripts might be considered as a strategy to mitigate the onset of CCA in PSC patients, and warrants further experimental investigation. The analysis performed herein may be helpful in the identification of non-invasive biomarkers for the early detection of PSC and for predicting its progression into CCA. In conclusion, future clinical research should investigate in more depth the full potential of RBP transcripts as biomarkers for human pathologies.Copyright © 2024 Ala and Fagoonee.