Gene Therapy Center, University of Massachusetts Medical School, Worcester, MA 01605, USA.
Hum Gene Ther. 2013 May;24(5):520-5. doi: 10.1089/hum.2012.112.
Recombinant adeno-associated viruses (rAAVs) have been tested in humans and other large mammals without adverse events. However, one study of mucopolysaccharidosis VII correction in mice showed repeated integration of rAAV in cells from hepatocellular carcinoma (HCC) in the Dlk1-Dio3 locus, suggesting possible insertional mutagenesis. In contrast, another study found no association of rAAV integration with HCC, raising questions about the generality of associations between liver transformation and integration at Dlk1-Dio3. Here we report that in rAAV-treated ornithine transcarbamylase (Otc)-deficient mice, four examples of integration sites in Dlk1-Dio3 could be detected in specimens from liver nodule/tumors, confirming previous studies of rAAV integration in the Dlk1-Dio3 locus in the setting of another murine model of metabolic disease. In one case, the integrated vector was verified to be present at about one copy per cell, consistent with clonal expansion. Another verified integration site in liver nodule/tumor tissue near the Tax1bp1 gene was also detected at about one copy per cell. The Dlk1-Dio3 region has also been implicated in human HCC and so warrants careful monitoring in ongoing human clinical trials with rAAV vectors.
重组腺相关病毒(rAAV)已在人体和其他大型哺乳动物中进行了测试,没有不良事件。然而,一项在小鼠中进行的黏多糖贮积症 VII 型校正研究表明,rAAV 会在 Dlk1-Dio3 基因座的肝细胞癌(HCC)细胞中反复整合,提示可能存在插入突变。相比之下,另一项研究发现 rAAV 整合与 HCC 之间没有关联,这引发了对 Dlk1-Dio3 整合与肝转化之间普遍关联的质疑。在这里,我们报告在接受 rAAV 治疗的鸟氨酸转氨甲酰酶(Otc)缺陷型小鼠中,在来自肝结节/肿瘤的标本中可以检测到 Dlk1-Dio3 中的四个整合位点,这证实了之前在另一种代谢疾病的小鼠模型中 rAAV 在 Dlk1-Dio3 基因座中的整合研究。在一种情况下,整合的载体被确认为每个细胞存在约一个拷贝,符合克隆扩张。在肝结节/肿瘤组织中靠近 Tax1bp1 基因的另一个已验证的整合位点也以每个细胞约一个拷贝的水平存在。Dlk1-Dio3 区域也与人类 HCC 有关,因此在正在进行的 rAAV 载体的人类临床试验中需要仔细监测。