Functional analysis of tonsil epithelial cells in children with PFAPA syndrome

2021 Fiscal Year Final Research Report

• Project/Area Number: 20K16910
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 52050:Embryonic medicine and pediatrics-related
• Research Institution: National Center for Child Health and Development
• Principal Investigator: Hara Mariko 国立研究開発法人国立成育医療研究センター, 免疫アレルギー・感染研究部, リサーチアソシエイト (30744552)
• Project Period (FY): 2020-04-01 – 2022-03-31
• Keywords: PFAPA症候群 / 扁桃上皮

Outline of Final Research Achievements

We examined the expression of genes and proteins induced in tonsillar epithelial cells during fever episodes of PFAPA syndrome and investigated what innate immunity-related factors are involved in fever. Transcriptome analysis showed altered expression of genes associated with PRRs and interferon signaling during fever episodes. In vitro stimulation of tonsillar epithelial cells indicated activation of pathways mediated by cytoplasmic RNA sensors. In addition, altered expression of CLDN5 and JAM-1 was observed by intracellular dsRNA stimuli. Our data suggested that intracellular dsRNA might cause inflammation and affect the barrier function of tonsillar epithelial cells in PFAPA syndrome.

Free Research Field

耳鼻咽喉科

Academic Significance and Societal Importance of the Research Achievements

PFAPA症候群は、扁桃炎を高率に随伴し、口蓋扁桃摘出術が著効する特徴をもっており、本疾患の炎症惹起には口蓋扁桃が深く関連していると推測されている。外来刺激に対する扁桃の免疫応答は、主に上皮細胞を介して行われるが、本疾患の扁桃上皮でどのような免疫応答が誘導されるかは分かっていない。本研究は、扁桃上皮における機能解析を行った研究であり、これまでに報告されていない内容である。