Hagstrom S A, Dryja T P
Ocular Molecular Genetics Institute, Harvard Medical School, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA.
Proc Natl Acad Sci U S A. 1999 Mar 16;96(6):2952-7. doi: 10.1073/pnas.96.6.2952.
Loss of heterozygosity at tumor-suppressor loci is an important oncogenic mechanism first discovered in retinoblastomas. We explored this phenomenon by examining a set of matched retinoblastoma and leukocyte DNA samples from 158 patients informative for DNA polymorphisms. Loss of heterozygosity at the retinoblastoma locus (13q14) was observed in 101 cases, comprising 7 cases with a somatic deletion causing hemizygosity and 94 with homozygosity (isodisomy). Homozygosity was approximately equally frequent in tumors from male and female patients, among patients with a germ-line vs. somatic initial mutation, and among patients in whom the initial mutation occurred on the maternal vs. paternal allele. A set of 75 tumors exhibiting homozygosity was investigated with markers distributed in the interval 13cen-13q14. Forty-one tumors developed homozygosity at all informative marker loci, suggesting that homozygosity occurred through chromosomal nondisjunction. The remaining cases exhibited mitotic recombination. There was no statistically significant bias in apparent nondisjunction vs. mitotic recombination among male vs. female patients or among patients with germ-line vs. somatic initial mutations. We compared the positions of somatic recombination events in the analyzed interval with a previously reported meiotic recombination map. Although mitotic crossovers occurred throughout the assayed interval, they were more likely to occur proximally than a comparable number of meiotic crossovers. Finally, we observed four triple-crossover cases, suggesting negative interference for mitotic recombination, the opposite of what is usually observed for meiotic recombination.
肿瘤抑制基因座杂合性缺失是一种重要的致癌机制,最早在视网膜母细胞瘤中被发现。我们通过检测158例具有DNA多态性信息的患者的一组匹配的视网膜母细胞瘤和白细胞DNA样本,对这一现象进行了探究。在101例病例中观察到视网膜母细胞瘤基因座(13q14)的杂合性缺失,其中7例存在导致半合子状态的体细胞缺失,94例存在纯合性(等臂双体)。在男性和女性患者的肿瘤中,在具有种系与体细胞初始突变的患者中,以及在初始突变发生在母本与父本等位基因上的患者中,纯合性的发生频率大致相同。使用分布在13cen - 13q14区间的标记对一组75例表现出纯合性的肿瘤进行了研究。41例肿瘤在所有信息性标记基因座上都出现了纯合性,表明纯合性是通过染色体不分离发生的。其余病例表现出有丝分裂重组。在男性与女性患者之间或在具有种系与体细胞初始突变的患者之间,明显的不分离与有丝分裂重组之间没有统计学上的显著偏差。我们将分析区间内体细胞重组事件的位置与先前报道的减数分裂重组图谱进行了比较。尽管有丝分裂交叉发生在整个检测区间,但它们比相当数量的减数分裂交叉更有可能发生在近端。最后,我们观察到4例三交叉病例,提示有丝分裂重组存在负干扰,这与通常在减数分裂重组中观察到的情况相反。