Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance

鎌田, 佳宏

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タイトル	Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance
別タイトル	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
タイトル (ヨミ)	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
著者	鎌田, 佳宏鎌田, 佳宏
著者 (ヨミ)	カマダ, ヨシヒロ
内容	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.
抄録	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.
著者所属研究科	医学系
専攻	生体制御医学
学位名	博士（医学）
学位授与年月日	2003-03-25
学位授与機関	大阪大学
学位授与番号	14401甲第08973号
学位記番号	17607
URL	http://hdl.handle.net/11094/2966
関連情報 (references)	http://www.jci.org/articles/view/11260
言語	英語

著者版フラグ	ETD
NII資源タイプ	学位論文
ローカル資源タイプ	博士論文本文あり
ローカル資源タイプ	博士論文
dcmi資源タイプ	text
DC.title	Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance
DCTERMS.alternative	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
DC.creator	鎌田, 佳宏
DC.language" scheme="DCTERMS.RFC1766	英語
DC.identifier" scheme="DCTERMS.URI	http://hdl.handle.net/11094/2966
DC.description	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.
DCTERMS.abstract	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.
DCTERMS.issued" scheme="DCTERMS.W3CDTF	2003-03-25
citation_title	Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance
citation_author	鎌田, 佳宏
citation_language	英語
citation_public_url	http://hdl.handle.net/11094/2966
citation_date	2003-03-25

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