L-アルギニン欠乏状態における血管内皮機能異常～リジン尿性蛋白不耐症患者における検討

• Material Type: 博士論文
• Title: L-アルギニン欠乏状態における血管内皮機能異常～リジン尿性蛋白不耐症患者における検討
• Title Transcription: L-アルギニン ケツボウジョウタイ ニオケル ケッカンナイヒ キノウイジョウ～リジン ニョウセイタンパク フタイショウカンジャ ニオケル ケントウ
• Author/Editor: 鎌田, 佳宏
• Author Heading: 鎌田, 佳宏 カマダ, ヨシヒロ; https://nrid.nii.ac.jp/nrid/1000030622609
• Alternative Title: Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance
• Degree grantor/type: 大阪大学
• Date Granted: 2003-03-25
• Dissertation Number: 甲第08973号
• Degree Type: 博士（医学）
• Text Language Code: eng
• Target Audience: 一般
• Note (General): Kamada, Yoshihiro; Nagaretani, Hiroyuki; Tamura, Shinji; Ohama, Tohru; Maruyama, Takao; Hiraoka, Hisatoyo; Yamashita, Shizuya; Yamada, Akira; Kiso, Shinichi; Inui, Yoshiaki; Ito, Nobuyuki; Kayanoki, Yoshiro; Kawata, Sumio; Matsuzawa, Yuji. Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance. Journal of Clinical Investigation. 2001. 108(5). p.717-724.; Although L-arginine is the only substrate for nitric oxide (NO) production, no studies have yet been reported on the effect of an L-arginine deficiency on vascular function in humans. Lysinuric protein intolerance (LPI) is a rare autosomal recessive defect of dibasic amino acid transport caused by mutations in the SLC7A7 gene, resulting in an L-arginine deficiency. Vascular endothelial function was examined in an LPI patient who was shown to be a compound heterozygote for two mutations in the gene (5.3-kbp Alu-mediated deletion, IVS3+1G→Α). The lumen diameter of the brachial artery was measured in this patient and in healthy controls at rest, during reactive hyperemia (endothelium-dependent vasodilation [EDV]), and after sublingual nitroglycerin administration (endothelium-independent vasodilation [EIV]) using ultrasonography. Both EDV and NOx concentrations were markedly reduced in the patient compared with those for the controls. They became normal after an L-arginine infusion. EIV was not significantly different between the patient and controls. Positron emission tomography of the heart and a treadmill test revealed ischemic changes in the patient, which were improved by the L-arginine infusion. Thus, in the LPI patient, L-arginine deficiency caused vascular endothelial dysfunction via a decrease in NO production.
• Format (IMT): application/pdf
• Access Restrictions: インターネット公開
• References (URI): http://www.jci.org/articles/view/11260; URI
• Data Provider (Database): 国立情報学研究所 : 学術機関リポジトリデータベース（IRDB）（機関リポジトリ）; https://irdb.nii.ac.jp
• Original Data Provider (Database): 大阪大学 : 大阪大学学術情報庫 桜華

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