Hu Caiqin, Zhu Biao
Department of Infectious Diseases, State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.
Zhejiang Da Xue Xue Bao Yi Xue Ban. 2018 May 25;47(5):534-540. doi: 10.3785/j.issn.1008-9292.2018.10.14.
Progressive multifocal leukoencephalopathy (PML) is a rare and lethal central nervous demyelinating disease caused by JC polyomavirus (JCV), particularly in patients with impaired immune system. The variation of JCV plays an important role in the pathogenesis of PML, including the recombination of non-coding regulatory region (NCCR), which is closely related to binding sites of transcription factors and affect the level of gene transcription. Nucleotide mutations in VP1 region determine the antigenicity and receptor specificity of JCV, play an important role in cell adsorption, immune-mediation and pathogenicity. In addition, immune cells are also involved in the pathogenesis of PML. T lymphocytes can recognize virus antigens, clear JCV, which are directly related to the prognosis of PML. B lymphocytes can serve as latent sites of JCV, and participate in viral transmission, replication, and coordination of the expression of transcription factors. This paper summarizes the roles of JCV variation and immune cells in pathogenesis of PML.
进行性多灶性白质脑病（PML）是一种由JC多瘤病毒（JCV）引起的罕见致命性中枢神经脱髓鞘疾病，尤其在免疫系统受损的患者中发病。JCV的变异在PML的发病机制中起重要作用，包括非编码调控区（NCCR）的重组，这与转录因子的结合位点密切相关并影响基因转录水平。VP1区域的核苷酸突变决定了JCV的抗原性和受体特异性，在细胞吸附、免疫介导和致病性方面起重要作用。此外，免疫细胞也参与了PML的发病机制。T淋巴细胞可识别病毒抗原，清除JCV，这与PML的预后直接相关。B淋巴细胞可作为JCV的潜伏位点，并参与病毒传播、复制以及转录因子表达的调控。本文总结了JCV变异和免疫细胞在PML发病机制中的作用。
