Tsuei Daw-Jen, Hsu Hey-Chi, Lee Po-Huang, Jeng Yung-Ming, Pu Yeong-Shiau, Chen Chiung-Nien, Lee Yung-Chie, Chou Wei-Chun, Chang Chung-Jung, Ni Yen-Hsuan, Chang Mei-Hwei
Department of Pediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taiwan.
Oncogene. 2004 Jul 29;23(34):5815-22. doi: 10.1038/sj.onc.1207773.
The RNA-binding motif (RRM) gene on Y chromosome (RBMY), encoding a male germ cell-specific RNA-binding protein associated with spermatogenesis, was found inserted by hepatitis B virus (HBV) DNA in one childhood hepatocellular carcinoma (HCC). This study is aimed to explore the oncogenic potential of the RBMY protein. The RBMY transcripts, expressed exclusively in the testis of normal people, were detected by reverse transcription-polymerase chain reaction in 36% of HCCs from 90 males and in 67% of hepatoblastoma from six boys. The nontumor liver counter parts, cirrhotic liver tissues from children with biliary atresia, and other types of cancers, such as bile duct, colon, stomach, lung, prostate, and kidney, were all negative for RBMY expression. One to four types of RBMY transcripts, including wild type and variants with N-terminal RRM deletion, C-terminal SRGY (serine-arginine-glycine-tyrosine) boxes deletion, or deletion of both domains, were found in the testis and liver cancer tissues. The wild-type RBMY protein was expressed in the nucleus and demonstrated its tumorigenicity by transformation of mouse fibroblast NIH3T3 cells and in vivo tumor formation. The RBMY variant protein with deletion of C-terminal exons 9-12 was trapped in the cytoplasm and showed decreased tumorigenicity. Our results suggest that RBMY is a new candidate oncogene specific for male liver cancer.
Y染色体上的RNA结合基序(RRM)基因(RBMY)编码一种与精子发生相关的雄性生殖细胞特异性RNA结合蛋白,在一例儿童肝细胞癌(HCC)中发现该基因被乙型肝炎病毒(HBV)DNA插入。本研究旨在探讨RBMY蛋白的致癌潜力。RBMY转录本仅在正常人睾丸中表达,通过逆转录聚合酶链反应在90名男性的36%的HCC和6名男孩的67%的肝母细胞瘤中检测到。非肿瘤肝脏对应物、胆管闭锁儿童的肝硬化肝组织以及其他类型的癌症,如胆管癌、结肠癌、胃癌、肺癌、前列腺癌和肾癌,RBMY表达均为阴性。在睾丸和肝癌组织中发现了一到四种类型的RBMY转录本,包括野生型以及N端RRM缺失、C端SRGY(丝氨酸-精氨酸-甘氨酸-酪氨酸)框缺失或两个结构域均缺失的变体。野生型RBMY蛋白在细胞核中表达,并通过转化小鼠成纤维细胞NIH3T3细胞和体内肿瘤形成证明其致瘤性。C端外显子9-12缺失的RBMY变体蛋白被困在细胞质中,致瘤性降低。我们的结果表明,RBMY是男性肝癌特有的一种新的候选癌基因。