| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Fiscal Year 2014: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
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| 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.
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