Genetic Epidemiology, Department of Internal Medicine, University of Utah, Salt Lake City, UT.
Department of Epidemiology, University of Texas MD Anderson Cancer Center, Houston, TX.
J Natl Cancer Inst. 2018 Dec 1;110(12):1380-1385. doi: 10.1093/jnci/djy058.
Statistically significant linkage of melanoma to chromosome 9q21 was previously reported in a Danish pedigree resource and independently confirmed in Utah high-risk pedigrees, indicating strong evidence that this region contains a melanoma predisposition gene.
Whole-exome sequencing of pairs of related melanoma case subjects from two pedigrees with evidence of 9q21 linkage was performed to identify the responsible predisposition gene. Candidate variants were tested for association with melanoma in an independent set of 454 unrelated familial melanoma case subjects and 396 unrelated cancer-free control subjects from Utah, and 1534 melanoma case subjects and 1146 noncancer control subjects from Texas (MD Anderson) via a two-sided Fisher exact test.
A rare nonsynonymous variant in Golgi Membrane Protein 1 (GOLM1), rs149739829, shared in two hypothesized predisposition carriers in one linked pedigree was observed. Segregation of this variant in additional affected relatives of the index carriers was confirmed. A statistically significant excess of carriers of the variant was observed among Utah case subjects and control subjects (odds ratio [OR] = 9.81, 95% confidence interval [CI] = 8.35 to 11.26, P < .001) and statistically significantly confirmed in Texas case subjects and control subjects (OR = 2.45, 95% CI = 1.65 to 3.25, P = .02).
These findings support GOLM1 as a candidate melanoma predisposition gene.
先前在丹麦家系资源中报道了黑色素瘤与染色体 9q21 之间存在统计学显著连锁,在犹他州高危家系中也独立得到证实,这表明该区域强烈提示存在黑色素瘤易感性基因。
对具有 9q21 连锁证据的两个家系中相关黑色素瘤病例对照进行全外显子组测序,以鉴定负责易感性的基因。候选变异通过双侧 Fisher 精确检验在来自犹他州的 454 例无关家族性黑色素瘤病例对照和 396 例无关癌症对照以及来自德克萨斯州(MD 安德森)的 1534 例黑色素瘤病例对照和 1146 例非癌症对照中进行与黑色素瘤的关联测试。
在一个连锁家系中两个假设的易感性携带者中观察到高尔基体膜蛋白 1(GOLM1)的罕见非同义变异 rs149739829。该变异在指数携带者的其他受影响亲属中的分离得到了确认。在犹他州病例对照和对照中观察到该变异携带者的比例显著增加(比值比[OR] = 9.81,95%置信区间[CI] = 8.35 至 11.26,P<.001),在德克萨斯州病例对照和对照中也得到了统计学显著证实(OR = 2.45,95% CI = 1.65 至 3.25,P =.02)。
这些发现支持 GOLM1 作为候选黑色素瘤易感性基因。