Analysis of the mechanism of break immune tolerance and blister formation in paraneoplastic pemphigus.

• Project/Area Number: 25860976
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Dermatology
• Research Institution: Kurume University
• Principal Investigator: NATSUAKI Yohei 久留米大学, 医学部, 助教 (40389309)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000); Fiscal Year 2014: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000); Fiscal Year 2013: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
• Keywords: 腫瘍随伴性天疱瘡 / 自己抗原 / A2ML1 / エピプラキン / 免疫ブロット法 / 免疫沈降法 / 閉塞性細気管支炎 / Paraneoplastic pemphigus / Epiplakin / Bronchiolitis obliterans

Outline of Final Research Achievements

We analyzed 108 cases of paraneoplastic pemphigus (PNP) in detail. We indicated the possibility that autoantibody for A2ML1 which is newly specific autoantigen was one of the pathogenic autoantibody of PNP. Furthermore, we indicated the possibility of the autoantibody production from associated tumor. Additionally, we indicated that epiplakin (EPPK) which is a plakin protein was one of the major PNP pathogenetic autoantigens and was related to PNP-related bronchiolitis obliterans (BO). The presence of anti-EPPK antibodies was significantly related to the development of BO and higher mortality. Therefore, detection of anti-EPPK antibodies may lead to more extensive treatments to avoid the development of fatal PNP-related BO.

Research Products

• [Journal Article] Epiplakin Is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients. (2016)
  • Author(s): Tsuchisaka A, Numata S, Teye K, et al.
  • Journal Title: J Invest Dermatol Volume: 136 Issue: 2 Pages: 399-408
  • DOI: 10.1038/jid.2015.408
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Anti-alpha-2-macroglobulin-like-1 Autoantibodies are Detected Frequently and may be Pathogenic in Paraneoplastic Pemphigus. (2013)
  • Author(s): Numata S, Teye K, Tsuruta D, Sogame R, Ishii N, Koga H, Natsuaki Y, Tsuchisaka A, Hamada T, Karashima T,Nakama T, Furumura M, Ohata C, Kawakami T, Schepens I, Borradori L, Hashimoto T.
  • Journal Title: J Invest Dermatol Volume: Epub ahead of print Issue: 7 Pages: 1785-1793
  • DOI: 10.1038/jid.2013.65
  • Peer Reviewed