Assignment of Six Patients with Xeroderma Pigmentosum in Hokkaido Area to A Variant Form
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概要
- 論文の詳細を見る
Six Japanese patients in Hokkaido area were diagnosed as xeroderma pigmentosum (XP) at two University Hospitals; one patient, a 29 years old female (XP2AS) at Asahikawa Medical College, and five patients, namely, a 53 years old male (XP1SA), a 45 years old female (XP2SA), a 46 years old female (XP3SA), a 47 years old male (XP4SA) and a 34 years old male (XP5SA) at Hokkaido University School of Medicine. All these XP patients showed mild skin symptoms and had no apparent neurological abnormalities. To identify genetic complementation groups of these patients, DNA repair capacities in skin fibroblasts derived from the patients were analyzed by ultraviolet light (UV)-induced unscheduled DNA synthesis (UDS), and also by colony-forming abilities of UV-irradiated cells incubated with or without caffeine added in post-UV culture medium. The levels of UDS measured by autoradiography in these XP cells irradiated with 20 J/m^2 of UV were 67 to 84% of those of normal human fibroblasts. Although one XP cell strain (XP2AS) showed about 2-fold greater sensitivity to killing by UV than normal cells, UV sensitivities of the rest of 5 XP strains were similar or only slightly higher than those of normal cells. However, UV sensitivities of all the 6 XP cell strains markedly increased by addition of I mM caffeine in the post-UV incubation medium, a phenomenon characteristic of XP variant cells. We also analyzed post-replication repair in XPISA cells by sedimentation of newly-synthesized DNA in alkaline sucrose gradient, and found that this strain is defective in this repair system. From these results, we concluded that all the 6 XP strains studied here belong to XP variants.
- 日本放射線影響学会の論文
著者
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IIZUKA Hajime
Department of Dermatology, Asahikawa Medical College
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池永 満生
京大・放生研
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TAKATERA HIROSHI
Department of Radiation Biology, Faculty of Medicine, Osaka University
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池永 満生
関西外国語大学
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池永 満生
京都大学放射線生物研究センター
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FUJIKAWA KAYO
京都大学放射線生物研究センター
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AYAKI HITOSHI
京都大学放射線生物研究センター
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ISHIZAKI KANJI
京都大学放射線生物研究センター
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MATSUO SHINOBU
Department of Dermatology, Asahikawa Medical College
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KOIZUMI HIROKO
Department of Dermatology, Hokkaido University School of Medicine
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池永 満生
京大 放射線生物研セ
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Matsuo Shinobu
Department Of Dermatology Asahikawa Medical College
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Matsuo Shinobu
Department Of Bio-medical Engineering Tokai University
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Iizuka Hajime
Department Of Dermatology Asahikawa Medical College
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Koizumi Hiroko
Department Of Applied Chemistry Faulty Of Engineering Kogakuin University
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Takatera Hiroshi
Department Of Radiation Biology Faculty Of Medicine Osaka University
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