The Gene Structure of the 450-kDa Autoantigen and its Regulation
| Project/Area Number |
11670839
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Dermatology
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| Research Institution | Oita Medical University |
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Principal Investigator |
FUJIWARA Sakuhei Oita Medical University, School of Medicine, Associate Professor, 医学部, 助教授 (90181411)
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| Co-Investigator(Kenkyū-buntansha) |
OKAMOTO Osamu Oita Medical University, School of Medicine, Assistant, 医学部, 助手 (40284799)
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| Project Period (FY) |
1999 – 2000
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| Project Status |
Completed (Fiscal Year 2000)
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| Budget Amount *help |
¥3,400,000 (Direct Cost: ¥3,400,000)
Fiscal Year 2000: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 1999: ¥2,800,000 (Direct Cost: ¥2,800,000)
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| Keywords | Bullous Pemphigoid / plectin / hemidesmosome / plakin family / keratinocyte / intermediate filament / cell attachment / autoantigen |
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| Research Abstract |
A 450-kDa human epidermal autoantigen was originally identified as a protein that reacted with the serum from an individual with a subepidermal blistering disease. Molecular cloning of this protein has now shown that it contains 5,065 amino acids and has a molecular mass of 552kDa. As reported previously this protein, which we call epiplakin, belongs to the plakin family, but it has some very unusual features. Epiplakin has 13 domains that are homologous to the B domain in the carboxy-terminal region of desmoplakin. The last five of these B domains, together with their associated linker regions, are particularly strongly conserved. However, epiplakin lacks a coiled-coil rod domain and an amino-terminal domain, both of which are found in all other known members of the plakin family. Furthermore, no dimerization motif were found in the sequence. Thus, it is likely that epiplakin exists in vivo as a single-chain structure. Epitope mapping experiments showed that the original patient's serum recognized a sequence unique to epiplakin, which was not found in plectin. Immunofluorescence staining revealed the presence of epiplakin in whole sheets of epidermis and esophagus, in glandular cells of eccrine sweat and parotid glands and in mucous epithelial cells in the stomach and colon.
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Report
(3 results)
Research Products
(10 results)
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[Publications] Fujiwara.S, Takeo.N, Otani.Y, Parry.D.A.D., Kunimatsu.M, Lu.R, Sasaki.M, Matsuo.N, Khaleduzzaman.M, Yoshioka.H: "Epiplakin, a Novel Member of the Plakin Family Originally Identified as a 450-kDa Human Epidermal Autoantigen : Structure and Tissue Localization."J Biol Chem. (in press). (2001)
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