Martelli Maria Paola, Sozzi Gabriella, Hernandez Luis, Pettirossi Valentina, Navarro Alba, Conte Davide, Gasparini Patrizia, Perrone Federica, Modena Piergiorgio, Pastorino Ugo, Carbone Antonino, Fabbri Alessandra, Sidoni Angelo, Nakamura Shigeo, Gambacorta Marcello, Fernández Pedro Luis, Ramirez Jose, Chan John K C, Grigioni Walter Franco, Campo Elias, Pileri Stefano A, Falini Brunangelo
Institute of Hematology, University of Perugia, Perugia, Italy.
Am J Pathol. 2009 Feb;174(2):661-70. doi: 10.2353/ajpath.2009.080755. Epub 2009 Jan 15.
A fusion gene, echinoderm microtubule associated protein like 4-anaplastic lymphoma kinase (EML4-ALK), with transforming activity has recently been identified in a subset of non-small cell lung cancer (NSCLC), but its pathogenetic, diagnostic, and therapeutic roles remain unclear. Both frequency and type of EML4-ALK transcripts were investigated by reverse transcription PCR in 120 frozen NSCLC specimens from Italy and Spain; non-neoplastic lung tissues taken far from the tumor were used as controls. In cases carrying the fusion transcript, we determined EML4-ALK gene and protein levels using fluorescence in situ hybridization, Western blotting, and immunoprecipitation. We also analyzed ALK protein levels in paraffin samples from 662 NSCLC specimens, including the 120 cases investigated in the molecular studies. EML4-ALK transcripts (variants 1 and 3) were detected in 9 of 120 NSCLC samples but were not specific for NSCLC since they were also found in non-cancerous lung tissues taken far from the tumor. Notably, no transcripts were detected in matching tumor samples from these patients. Fluorescence in situ hybridization analysis of cases expressing EML4-ALK transcripts showed that only a minority of cells harbored the EML4-ALK gene. None of these cases was found to express the EML4-ALK protein as examined by immunohistochemistry, Western blotting, and immunoprecipitation. The EML4-ALK transcript cannot be regarded as a specific diagnostic tool for NSCLC. Our results show therefore that the causal role and value of EML4-ALK as a therapeutic target remain to be defined.
一种具有转化活性的融合基因,棘皮动物微管相关蛋白样4-间变性淋巴瘤激酶(EML4-ALK),最近在一部分非小细胞肺癌(NSCLC)中被发现,但其致病、诊断和治疗作用仍不清楚。通过逆转录PCR对来自意大利和西班牙的120份冷冻NSCLC标本中EML4-ALK转录本的频率和类型进行了研究;取自远离肿瘤的非肿瘤性肺组织用作对照。在携带融合转录本的病例中,我们使用荧光原位杂交、蛋白质印迹和免疫沉淀法测定了EML4-ALK基因和蛋白水平。我们还分析了662份NSCLC标本石蜡样本中的ALK蛋白水平,其中包括分子研究中调查的120例病例。在120份NSCLC样本中的9份中检测到了EML4-ALK转录本(变体1和3),但它们并非NSCLC所特有,因为在远离肿瘤的非癌性肺组织中也发现了它们。值得注意的是,在这些患者的匹配肿瘤样本中未检测到转录本。对表达EML4-ALK转录本的病例进行荧光原位杂交分析表明,只有少数细胞含有EML4-ALK基因。通过免疫组织化学、蛋白质印迹和免疫沉淀检测,这些病例均未发现表达EML4-ALK蛋白。EML4-ALK转录本不能被视为NSCLC的特异性诊断工具。因此,我们的结果表明,EML4-ALK作为治疗靶点的因果作用和价值仍有待确定。