School of Veterinary Medicine and Science, Sutton Bonington Campus, University of Nottingham , Leicestershire , UK.
School of Veterinary Medicine and Science, Sutton Bonington Campus, University of Nottingham , Leicestershire , UK ; Advanced Data Analysis Centre, Sutton Bonington Campus, University of Nottingham , Leicestershire , UK.
PeerJ. 2015 Mar 26;3:e842. doi: 10.7717/peerj.842. eCollection 2015.
Dilated cardiomyopathy is a prevalent and often fatal disease in humans and dogs. Indeed dilated cardiomyopathy is the third most common form of cardiac disease in humans, reported to affect approximately 36 individuals per 100,000 individuals. In dogs, dilated cardiomyopathy is the second most common cardiac disease and is most prevalent in the Irish Wolfhound, Doberman Pinscher and Newfoundland breeds. Dilated cardiomyopathy is characterised by ventricular chamber enlargement and systolic dysfunction which often leads to congestive heart failure. Although multiple human loci have been implicated in the pathogenesis of dilated cardiomyopathy, the identified variants are typically associated with rare monogenic forms of dilated cardiomyopathy. The potential for multigenic interactions contributing to human dilated cardiomyopathy remains poorly understood. Consistent with this, several known human dilated cardiomyopathy loci have been excluded as common causes of canine dilated cardiomyopathy, although canine dilated cardiomyopathy resembles the human disease functionally. This suggests additional genetic factors contribute to the dilated cardiomyopathy phenotype.This study represents a meta-analysis of available canine dilated cardiomyopathy genetic datasets with the goal of determining potential multigenic interactions relating the sex chromosome genotype (XX vs. XY) with known dilated cardiomyopathy associated loci on chromosome 5 and the PDK4 gene in the incidence and progression of dilated cardiomyopathy. The results show an interaction between known canine dilated cardiomyopathy loci and an unknown X-linked locus. Our study is the first to test a multigenic contribution to dilated cardiomyopathy and suggest a genetic basis for the known sex-disparity in dilated cardiomyopathy outcomes.
扩张型心肌病是人类和犬类中一种常见且通常致命的疾病。事实上,扩张型心肌病是人类中第三大常见的心脏病形式,据报道,每 10 万人中约有 36 人患有该病。在犬类中,扩张型心肌病是第二大常见的心脏病,在爱尔兰猎狼犬、杜宾犬和纽芬兰犬中最为常见。扩张型心肌病的特征是心室腔扩大和收缩功能障碍,这通常导致充血性心力衰竭。尽管已经有多个人类基因座被牵连到扩张型心肌病的发病机制中,但已确定的变体通常与罕见的单基因形式的扩张型心肌病相关。多基因相互作用对人类扩张型心肌病的潜在影响仍知之甚少。与此一致的是,几个已知的人类扩张型心肌病基因座已被排除为犬扩张型心肌病的常见原因,尽管犬扩张型心肌病在功能上与人类疾病相似。这表明其他遗传因素也导致了扩张型心肌病表型。
本研究对现有的犬扩张型心肌病遗传数据集进行了荟萃分析,目的是确定与性染色体基因型(XX 与 XY)相关的多基因相互作用,以及与已知的扩张型心肌病相关的染色体 5 上的基因座和 PDK4 基因在扩张型心肌病的发病和进展中的潜在关系。结果显示,已知的犬扩张型心肌病基因座与一个未知的 X 连锁基因座之间存在相互作用。我们的研究首次测试了多基因对扩张型心肌病的贡献,并为已知的扩张型心肌病性别差异提供了遗传基础。