Abstract：
BACKGROUND: MicroRNAs (miRNAs) are a group of small non-coding RNAs that bind to the target mRNA and regulate gene expression. Recently circulating microRNAs were investigated as markers of diseases and therapeutic targets. Although various studies analyze the miRNA expression in liver disease, these studies on PFIC are few. Progressive familial intrahepatic cholestasis (PFIC) is a rare liver disease with autosomal recessive inheritance. Most children with PFIC progress to cirrhosis and liver failure and consequently need to have a liver transplant. The aim of this study is the investigation of the miR-19b and miR-let7b expression levels in Iranian PFIC children.
METHODS: 25 PFIC patients, 25 healthy children and 25 Biliary Atresia patients were considered as case and two control groups respectively. Blood samples were obtained and Liver function tests (LFTs) were measured. After RNA extraction and cDNA synthesis, quantitative PCR was performed using specific primers for miR-19b and miR-let7b. The U6 gene is used as an internal control.
RESULTS: qPCR on PFIC patients\' samples demonstrated that the miR-19b and the miR-let7b expression were significantly decreased in patients compared to the control groups, with a p-value<0.0001 and p-value=0.0006 receptively.
CONCLUSIONS: In conclusion, circulating micro-RNA like miR-19b and miR-let7b have a potential opportunity to be a non-invasive diagnostic marker or therapeutic target for PFIC in the future.
METHODS: 25 PFIC patients, 25 healthy children and 25 Biliary Atresia patients were considered as case and two control groups respectively. Blood samples were obtained and Liver function tests (LFTs) were measured. After RNA extraction and cDNA synthesis, quantitative PCR was performed using specific primers for miR-19b and miR-let7b. The U6 gene is used as an internal control.
RESULTS: qPCR on PFIC patients\' samples demonstrated that the miR-19b and the miR-let7b expression were significantly decreased in patients compared to the control groups, with a p-value<0.0001 and p-value=0.0006 receptively.
CONCLUSIONS: In conclusion, circulating micro-RNA like miR-19b and miR-let7b have a potential opportunity to be a non-invasive diagnostic marker or therapeutic target for PFIC in the future.
摘要：
背景：微RNA（miRNA）是一组与靶mRNA结合并调节基因表达的小的非编码RNA。最近循环的microRNA作为疾病和治疗靶标的标志物被研究。尽管各种研究分析了miRNA在肝脏疾病中的表达，这些关于PFIC的研究很少。进行性家族性肝内胆汁淤积（PFIC）是一种罕见的常染色体隐性遗传肝病。大多数患有PFIC的儿童进展为肝硬化和肝功能衰竭，因此需要进行肝移植。这项研究的目的是调查伊朗PFIC儿童中miR-19b和miR-let7b的表达水平。
方法：25名PFIC患者，25例健康儿童和25例胆道闭锁患者分别作为病例组和两个对照组。获得血液样品并测量肝功能测试(LFTs)。RNA提取和cDNA合成后，使用miR-19b和miR-let7b的特异性引物进行定量PCR。U6基因用作内部对照。
结果：对PFIC患者\'样本的qPCR表明，与对照组相比，患者的miR-19b和miR-let7b表达显着降低，p值<0.0001，p值=0.0006。
结论：结论：循环微小RNA,如miR-19b和miR-let7b,未来有可能成为PFIC的非侵入性诊断标记或治疗靶点.
方法：25名PFIC患者，25例健康儿童和25例胆道闭锁患者分别作为病例组和两个对照组。获得血液样品并测量肝功能测试(LFTs)。RNA提取和cDNA合成后，使用miR-19b和miR-let7b的特异性引物进行定量PCR。U6基因用作内部对照。
结果：对PFIC患者\'样本的qPCR表明，与对照组相比，患者的miR-19b和miR-let7b表达显着降低，p值<0.0001，p值=0.0006。
结论：结论：循环微小RNA,如miR-19b和miR-let7b,未来有可能成为PFIC的非侵入性诊断标记或治疗靶点.
