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J-GLOBAL ID：201902222121449484   Reference number：19A1861487

Abnormal Amino Acid Profiles of Blood and Cerebrospinal Fluid from Cystathionine β-Synthase-Deficient Mice, an Animal Model of Homocystinuria

ホモシスチン尿症の動物モデルであるシスタチオニンβ-シンターゼ欠損マウスの血液および脳脊髄液の異常アミノ酸プロファイル

Author (8)： Akahoshi Noriyuki (Department of Health Chemistry, Showa Pharmaceutical University) ,  Yokoyama Akira (Department of Health Chemistry, Showa Pharmaceutical University) ,  Nagata Tomoko (Department of Biochemistry, Keio University Faculty of Pharmacy) ,  Miura Asumi (Department of Biochemistry, Keio University Faculty of Pharmacy) ,  Kamata Shotaro (Department of Health Chemistry, Showa Pharmaceutical University) ,  Kamata Shotaro (Department of Biochemistry, Keio University Faculty of Pharmacy) ,  Ishii Isao (Department of Health Chemistry, Showa Pharmaceutical University) ,  Ishii Isao (Department of Biochemistry, Keio University Faculty of Pharmacy)
Material： Biological and Pharmaceutical Bulletin (Web)
Volume： 42  Issue： 6  Page： 1054-1057(J-STAGE)  Publication year： 2019 
JST Material Number： U1402A  ISSN： 1347-5215  Document type： Article
Article type： 原著論文  Country of issue： Japan (JPN)  Language： ENGLISH (EN)

Thesaurus term： Mus musculus ,...
Semi thesaurus term： *Homocystinuria

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Author keywords (12)： homocystinuria ,  amino acid ,  cerebrospinal fluid ,  homocysteine ,  cystathionine β-synthase ,  mental retardation ,  homocystinuria ,  amino acid ,  cerebrospinal fluid ,  homocysteine ,  cystathionine β-synthase ,  mental retardation

JST classification (1)： Metabolic diseases,nutritional diseases in general.  (GD05010J)

Substance index (11)： Threonine ,  Asparagine ,  Aspartic Acid ,  Glutamine ,  Citrulline ,  Serine ,  Tyrosine ,  Phenylalanine ,  Homocysteine ,  Methionine ,  Lysine

Reference (17)：

• 1) Carson NA, Cusworth DC, Dent CE, Field CM, Neill DW, Westall RG. Homocystinuria: a new inborn error of metabolism associated with mental deficiency. Arch. Dis. Child., 38, 425-436 (1963).
• 2) Mudd SH, Levy HL, Kraus JP. Disorders of transsulfuration. The Metabolic and Molecular Bases for Inherited Disease. (Sciver CR, Beaudet AL, Sly WS, Valle D ed.) 8th Ed., McGraw-Hill, New York, pp. 2007-2056 (2001).
• 3) Morris AA, Kozich V, Santra S, Andria G, Ben-Omran TI, Chakrapani AB, Crushell E, Henderson MJ, Hochuli M, Huemer M, Janssen MC, Maillot F, Mayne PD, McNulty J, Morrison TM, Ogier H, O’Sullivan S, Pavlikova M, de Almeida IT, Terry A, Yap S, Blom HJ, Chapman KA. Guidelines for the diagnosis and management of cystathionine beta-synthase deficiency. J. Inherit. Metab. Dis., 40, 49-74 (2017).
• 4) Lai WK, Kan MY. Homocysteine-induced endothelial dysfunction. Ann. Nutr. Metab., 67, 1-12 (2015).
• 5) Ganguly P, Alam SF. Role of homocysteine in the development of cardiovascular disease. Nutr. J., 14, 6 (2015).

Terms in the title (10)： ホモシスチン尿症 ,  動物 ,  モデル ,  シスタチオニンβ-シンターゼ ,  欠損 ,  マウス ,  血液 ,  脳脊髄液 ,  アミノ酸 ,  プロファイル