Department of Pathology and Cambridge Institute for Medical Research, University of Cambridge, Cambridge CB2 1QP, United Kingdom; email:
Annu Rev Genomics Hum Genet. 2013;14:301-23. doi: 10.1146/annurev-genom-091212-153455. Epub 2013 Jul 15.
Over several decades, various forms of genomic analysis of the human major histocompatibility complex (MHC) have been extremely successful in picking up many disease associations. This is to be expected, as the MHC region is one of the most gene-dense and polymorphic stretches of human DNA. It also encodes proteins critical to immunity, including several controlling antigen processing and presentation. Single-nucleotide polymorphism genotyping and human leukocyte antigen (HLA) imputation now permit the screening of large sample sets, a technique further facilitated by high-throughput sequencing. These methods promise to yield more precise contributions of MHC variants to disease. However, interpretation of MHC-disease associations in terms of the functions of variants has been problematic. Most studies confirm the paramount importance of class I and class II molecules, which are key to resistance to infection. Infection is likely driving the extreme variation of these genes across the human population, but this has been difficult to demonstrate. In contrast, many associations with autoimmune conditions have been shown to be specific to certain class I and class II alleles. Interestingly, conditions other than infections and autoimmunity are also associated with the MHC, including some cancers and neuropathies. These associations could be indirect, owing, for example, to the infectious history of a particular individual and selective pressures operating at the population level.
几十年来,对人类主要组织相容性复合体(MHC)的各种形式的基因组分析在发现许多疾病相关性方面取得了巨大成功。这是意料之中的,因为 MHC 区域是人类 DNA 中基因密度和多态性最高的区域之一。它还编码对免疫至关重要的蛋白质,包括几个控制抗原加工和呈递的蛋白质。单核苷酸多态性基因分型和人类白细胞抗原(HLA)推断现在允许对大样本集进行筛选,高通量测序进一步促进了这项技术。这些方法有望更准确地确定 MHC 变异对疾病的贡献。然而,根据变异体的功能来解释 MHC 与疾病的关联一直存在问题。大多数研究证实了 I 类和 II 类分子的首要重要性,这些分子是抵抗感染的关键。感染很可能是导致这些基因在人类群体中发生极端变异的原因,但这很难证明。相比之下,许多与自身免疫性疾病相关的关联被证明是特定于某些 I 类和 II 类等位基因的。有趣的是,除了感染和自身免疫性疾病外,MHC 还与其他疾病有关,包括某些癌症和神经病变。这些关联可能是间接的,例如,由于个体的特定感染史和人群水平上的选择压力。