Zhu Siying, Tao Hongxu, Cornell Robert A, Liu Huan
State Key Laboratory of Oral & Maxillofacial Reconstruction and Regeneration, Key Laboratory of Oral Biomedicine Ministry of Education, Hubei Key Laboratory of Stomatology, School & Hospital of Stomatology, Wuhan University, Wuhan, China.
Department of Oral Health Sciences, School of Dentistry, University of Washington, Seattle, Washington, USA.
Hum Mutat. 2025 Aug 28;2025:6824122. doi: 10.1155/humu/6824122. eCollection 2025.
Over the past decade, genome-wide association studies (GWASs) have found genetic variants associated with elevated risk for nonsyndromic orofacial cleft (NSOFC). In the post-GWAS era of NSOFC genetic research, an important aim is to identify the pathogenic variants that influence craniofacial development processes, towards understanding how they lead to disease manifestation. However, two major challenges hinder the translation of GWAS results into a mechanistic understanding. Firstly, it is uncertain whether the variants pinpointed by GWAS represent the underlying pathogenic variants; secondly, the bulk of genetic variants identified through GWAS are situated in noncoding regions of the genome, complicating their biological interpretation. Presently, research on noncoding genetic variants associated with NSOFC predominantly centers on variants located in transcriptional regulatory elements. These variants modulate transcription, subsequently altering the expression of downstream target genes and disrupting gene regulatory networks. We provide a systematic summary of the recent NSOFC-associated GWAS findings for the first time. With a particular focus on variants located in noncoding regions, we delve into current statistical methods and functional approaches for identifying and validating causal variants, aiming to bridge the gap between genetic variants identified by GWAS and their underlying pathogenic mechanism responsible for NSOFC. Deciphering causal variants underlying NSOFC offers valuable clinical insights that may advance early diagnosis, enhance risk stratification, and facilitate the discovery of novel therapeutic targets.
在过去十年中,全基因组关联研究(GWAS)已经发现了与非综合征性口面部裂隙(NSOFC)风险升高相关的基因变异。在NSOFC基因研究的GWAS后时代,一个重要目标是识别影响颅面发育过程的致病变异,以了解它们如何导致疾病表现。然而,两个主要挑战阻碍了将GWAS结果转化为机制性理解。首先,GWAS确定的变异是否代表潜在的致病变异尚不确定;其次,通过GWAS鉴定的大部分基因变异位于基因组的非编码区域,这使得它们的生物学解释变得复杂。目前,与NSOFC相关的非编码基因变异研究主要集中在位于转录调控元件中的变异。这些变异调节转录,随后改变下游靶基因的表达并破坏基因调控网络。我们首次对最近与NSOFC相关的GWAS发现进行了系统总结。特别关注位于非编码区域的变异,我们深入研究了用于识别和验证因果变异的当前统计方法和功能方法,旨在弥合GWAS识别的基因变异与其导致NSOFC的潜在致病机制之间的差距。解读NSOFC潜在的因果变异提供了有价值的临床见解,可能会推动早期诊断、加强风险分层并促进新治疗靶点的发现。