PMID- 27467284 OWN - NLM STAT- MEDLINE DCOM- 20170518 LR - 20181113 IS - 1476-5470 (Electronic) IS - 1466-4879 (Linking) VI - 17 IP - 6 DP - 2016 Sep TI - Specific HLA genotypes confer susceptibility to acute necrotizing encephalopathy. PG - 367-9 LID - 10.1038/gene.2016.32 [doi] AB - Acute necrotizing encephalopathy (ANE) is a rare and severe syndrome of acute encephalopathy triggered by viral infections. Cytokine storm is considered as the main pathogenetic mechanism of ANE. ANE is prevalent in East Asia, suggesting the association of host genetic factors. To elucidate the genetic background of Japanese ANE, we examined genotypes of human leukocyte antigen (HLA)-A, C, B, DRB1, DQB1 and DPB1 in 31 patients. Significant positive association was observed in both the allele frequency and positivity of DRB1*09:01 (P=0.043 and 0.025, respectively), as well as those of DQB1*03:03 (P=0.034 and 0.026, respectively). The carrier frequency of DRB1*09:01 and DQB1*03:03 alleles was higher in the patients (45.16%) than in controls (28.57%). These alleles are more common in East Asian than in European populations, and are reportedly associated with various autoimmune diseases in Japanese patients. Our data provide further evidence that altered immune response based on individual HLA genotypes may contribute to ANE pathogenesis. FAU - Hoshino, A AU - Hoshino A AD - Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. AD - Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. FAU - Saitoh, M AU - Saitoh M AD - Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. FAU - Miyagawa, T AU - Miyagawa T AD - Department of Human Genetics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. AD - Sleep Disorders Project, Department of Psychiatry and Behavioral Sciences, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan. FAU - Kubota, M AU - Kubota M AD - Division of Neurology, National Center for Child Health and Development, Tokyo, Japan. FAU - Takanashi, J-I AU - Takanashi JI AD - Department of Pediatrics, Tokyo Women's Medical University Yachiyo Medical Center, Yachiyo, Japan. FAU - Miyamoto, A AU - Miyamoto A AD - Department of Pediatrics, Asahikawa Habilitation Center for Disabled Children, Asahikawa, Japan. FAU - Tokunaga, K AU - Tokunaga K AD - Department of Human Genetics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. FAU - Oka, A AU - Oka A AD - Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. FAU - Mizuguchi, M AU - Mizuguchi M AD - Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160728 PL - England TA - Genes Immun JT - Genes and immunity JID - 100953417 RN - 0 (HLA Antigens) SB - IM MH - Alleles MH - Disease Susceptibility MH - Encephalitis, Viral/*genetics/pathology MH - Genetic Predisposition to Disease MH - Genotype MH - HLA Antigens/*genetics MH - Humans MH - Leukoencephalitis, Acute Hemorrhagic/*genetics EDAT- 2016/07/29 06:00 MHDA- 2017/05/19 06:00 CRDT- 2016/07/29 06:00 PHST- 2016/05/19 00:00 [received] PHST- 2016/06/21 00:00 [revised] PHST- 2016/06/23 00:00 [accepted] PHST- 2016/07/29 06:00 [entrez] PHST- 2016/07/29 06:00 [pubmed] PHST- 2017/05/19 06:00 [medline] AID - gene201632 [pii] AID - 10.1038/gene.2016.32 [doi] PST - ppublish SO - Genes Immun. 2016 Sep;17(6):367-9. doi: 10.1038/gene.2016.32. Epub 2016 Jul 28.