Child Language Doctoral Program (CLDP), University of Kansas, Lawrence, KS, USA.
Institute of Chemistry, University of the Punjab, Lahore, Pakistan.
Eur J Hum Genet. 2019 Aug;27(8):1274-1285. doi: 10.1038/s41431-019-0398-1. Epub 2019 Apr 11.
Language is a uniquely human ability, and failure to attain this ability can have a life-long impact on the affected individuals. This is particularly true for individuals with specific language impairment (SLI), which is defined as an impairment in normal language development in the absence of any other developmental disability. Although SLI displays high heritability, family-based linkage studies have been hampered by an unclear mode of Mendelian segregation, variable disease penetrance, and heterogeneity of diagnostic criteria. We performed genome-wide parametric linkage analysis and homozygosity mapping in 14 consanguineous families from Pakistan segregating SLI. Linkage analysis revealed a multipoint LOD score of 4.18 at chromosome 2q in family PKSLI05 under a recessive mode of inheritance. A second linkage score of 3.85 was observed in family PKSLI12 at a non-overlapping locus on chromosome 2q. Two other suggestive linkage loci were found in family PKSLI05 on 14q and 22q with LOD scores of 2.37 and 2.23, respectively, that were also identified in homozygosity mapping. Reduction to homozygosity was observed on chromosomes 2q, 5p, 8q, 14q, 17q, and 22q. Each homozygosity region occurred in multiple PKSLI families. We report new SLI loci on chromosomes 2 and 8 and confirm suggestive SLI linkage loci on chromosomes 5, 14, 17, and 22 reported previously in the population of Robinson Crusoe Island. These findings indicate that linkage and homozygosity mapping in consanguineous families can improve genetic analyses in SLI and suggest the involvement of additional genes in the causation of this disorder.
语言是人类独有的能力,无法获得这种能力会对受影响的个体产生终身影响。对于特定语言障碍(SLI)患者来说尤其如此,SLI 定义为在没有任何其他发育障碍的情况下,正常语言发育受损。尽管 SLI 表现出高度的遗传性,但基于家族的连锁研究受到孟德尔分离模式不明确、疾病外显率可变和诊断标准异质性的阻碍。我们对来自巴基斯坦的 14 个 SLI 分离的近亲家庭进行了全基因组参数连锁分析和纯合子作图。连锁分析显示,在以隐性遗传模式遗传的 PKSLI05 家族中,2 号染色体上的多点 LOD 得分为 4.18。在不重叠的 2 号染色体位置上,PKSLI12 家族观察到第二个 3.85 的连锁得分。在 PKSLI05 家族中,还在 14 号染色体和 22 号染色体上发现了另外两个提示性连锁基因座,其 LOD 得分分别为 2.37 和 2.23,这些基因座在纯合子作图中也被发现。在染色体 2q、5p、8q、14q、17q 和 22q 上观察到纯合性减少。每个纯合区域都出现在多个 PKSLI 家族中。我们报告了染色体 2 和 8 上的新 SLI 基因座,并确认了先前在鲁滨逊克鲁索岛人群中报道的染色体 5、14、17 和 22 上的提示性 SLI 连锁基因座。这些发现表明,在近亲家庭中进行连锁和纯合子作图可以改善 SLI 的遗传分析,并表明该疾病的发病机制涉及其他基因。