Finette Barry A
Department of Pediatrics, Microbiology and Molecular Genetics, University of Vermont College of Medicine, E203 Given Building, 89 Beaumont Ave., Burlington, VT 05405, USA.
DNA Repair (Amst). 2006 Sep 8;5(9-10):1049-64. doi: 10.1016/j.dnarep.2006.05.023. Epub 2006 Jun 27.
Pediatric acute lymphocytic leukemia (ALL) is a multifactorial malignancy with many distinctive developmentally specific features that include age specific acquisition of deletions, insertions and chromosomal translocations. The analysis of breakpoint regions involved in these leukemogenic genomic rearrangements has provided evidence that many are the consequence of V(D)J recombinase mediated events at both immune and non-immune loci. Hence, the direct investigation of in vivo genetic and epigenetic features in human peripheral lymphocytes is necessary to fully understand the mechanisms responsible for the specificity and frequency of these leukemogenic non-immune V(D)J recombinase events. In this review, I will present the utility of analyzing mutagenic V(D)J recombinase mediated genomic rearrangements at the HPRT locus in humans as an in vivo model system for understanding the mechanisms responsible for leukemogenic genetic alterations observed in children with leukemia.
小儿急性淋巴细胞白血病（ALL）是一种多因素恶性肿瘤，具有许多独特的发育特异性特征，包括特定年龄时出现的缺失、插入和染色体易位。对这些致白血病基因组重排所涉及的断点区域进行分析，结果表明许多重排是免疫和非免疫基因座处V（D）J重组酶介导事件的结果。因此，要全面了解这些致白血病非免疫V（D）J重组酶事件的特异性和发生频率的机制，直接研究人类外周血淋巴细胞的体内遗传和表观遗传特征是必要的。在这篇综述中，我将介绍分析人类次黄嘌呤磷酸核糖转移酶（HPRT）基因座处诱变的V（D）J重组酶介导的基因组重排作为一种体内模型系统的作用，以了解白血病患儿中观察到的致白血病基因改变的机制。
