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

鎌田, 佳宏

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

鎌田, 佳宏

Number of Access:1,508（2025-12-09 03:40 Counts）

Identifier to cite or link to this item: https://hdl.handle.net/11094/2966

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17607_Dissertation	pdf	None	1.08 MB	226	2012.09.22	論文/Dissertation

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Title	Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance
	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
	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
Creator	NRID 1000030622609 1000030622609 ORCID 0000-0001-5485-902X 0000-0001-5485-902X researchmap permalink yk4478-jp yk4478-jp Scopus Author ID 7102943545 7102943545 CreatorName 鎌田, 佳宏 ; カマダ, ヨシヒロ
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.
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.
Degree Name	博士（医学）
Date of Granted	2003-03-25
Grantor	Osaka University
Faculty	医学系
Specialty	生体制御医学
Grant ID	甲第08973号
Description Number	17607
Language	English
Handle URL	https://hdl.handle.net/11094/2966
Relation.references	URI http://www.jci.org/articles/view/11260
Access Rights	open access
oaire:version	Version of Record
Category	Doctoral Thesis with Full Text / Graduate School of Medicine / 2002
Category	Doctoral Thesis / Graduate School of Medicine / 2002

Resource Type	doctoral thesis
Local Type	Doctoral Thesis with Full Text
Local Type	Doctoral Thesis
DCMI Type	Text
DCTERMS.bibliographicCitation	大阪大学, 2003, 博士論文
DC.title	Vascular endothelial dysfunction resulting from L-arginine deficiency in a patient with lysinuric protein intolerance
	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
	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
DC.creator	鎌田, 佳宏
DC.creator	カマダ, ヨシヒロ
DC.language' scheme='DCTERMS.RFC1766	English
DC.type' scheme='DCTERMS.DCMIType	Text
DC.relation' scheme='DCTERMS.URI	http://www.jci.org/articles/view/11260
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
DC.format	2966
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	English
citation_public_url	https://hdl.handle.net/11094/2966
citation_abstract_html_url	http://www.jci.org/articles/view/11260
citation_date	2003-03-25

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

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