Inflammatory cytokines and chemokines in an autoinflammatory disorder, Nakajo-Nishimura syndrome.

2015 Fiscal Year Final Research Report

• Project/Area Number: 25461478
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Collagenous pathology/Allergology
• Research Institution: Nagasaki University
• Principal Investigator: ARIMA Kazuhiko 長崎大学, 医歯薬学総合研究科(医学系), 講師 (30423635)
• Co-Investigator(Kenkyū-buntansha): IDA Hiroaki 久留米大学, 医学部, 教授 (60363496)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Keywords: 慢性炎症 / 炎症性疾患

Outline of Final Research Achievements

The ubiquitin-proteasome pathway is responsible for selective degradation of short-lived, misfolded and toxic proteins. It plays an important rule in transcriptional regulation. Nakajo-Nishimura syndrome (NNS) (MIM 256040) is an autosomal recessive autoinflammatory disorder characterized by periodic fever, partial lipodystrophy, contracture of joints, skin rash and calcification of basal ganglia. Homozygous mutation in the PSMB8 gene and decreased chymotrypsin-like activity indicated that dysfunction of the proteasome can result in the autoinflammatory condition. Genes were identified in cultured cells under the condition of proteasome inhibition. These findings suggest that decrease of proteasome activity is associated with an increased expression of inflammatory cytokines. The ubiquitin-proteasome pathway would play an important rule against inflammation. This may provide a new insight into the pathogenesis of other persistent inflammatory diseases.

Free Research Field

炎症学