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Homocystinuria: a new inborn error of metabolism associated with mental deficiency. Arch. Dis. Child.<\/i>, 38<\/b>, 425\u2013436 (1963)."},{"key":"2","unstructured":"2) Mudd SH, Levy HL, Kraus JP. Disorders of transsulfuration. The Metabolic and Molecular Bases for Inherited Disease.<\/i> (Sciver CR, Beaudet AL, Sly WS, Valle D ed.) 8th Ed., McGraw-Hill, New York, pp. 2007\u20132056 (2001)."},{"key":"3","doi-asserted-by":"crossref","unstructured":"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\u2019Sullivan 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.<\/i>, 40<\/b>, 49\u201374 (2017).","DOI":"10.1007\/s10545-016-9979-0"},{"key":"4","doi-asserted-by":"crossref","unstructured":"4) Lai WK, Kan MY. Homocysteine-induced endothelial dysfunction. Ann. Nutr. Metab.<\/i>, 67<\/b>, 1\u201312 (2015).","DOI":"10.1159\/000437098"},{"key":"5","doi-asserted-by":"crossref","unstructured":"5) Ganguly P, Alam SF. Role of homocysteine in the development of cardiovascular disease. Nutr. J.<\/i>, 14<\/b>, 6 (2015).","DOI":"10.1186\/1475-2891-14-6"},{"key":"6","doi-asserted-by":"crossref","unstructured":"6) Almuqbil MA, Waisbren SE, Levy HL, Picker JD. Revising the Psychiatric Phenotype of Homocystinuria. Genet. Med.<\/i>, (2019), in press.","DOI":"10.1038\/s41436-018-0419-4"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7) Akahoshi N, Kobayashi C, Ishizaki Y, Izumi T, Himi T, Suematsu M, Ishii I. Genetic background conversion ameliorates semi-lethality and permits behavioral analyses in cystathionine beta-synthase-deficient mice, an animal model for hyperhomocysteinemia. Hum. Mol. Genet.<\/i>, 17<\/b>, 1994\u20132005 (2008).","DOI":"10.1093\/hmg\/ddn097"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8) Ishii I, Akahoshi N, Yamada H, Nakano S, Izumi T, Suematsu M. Cystathionine gamma-lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury. J. Biol. Chem.<\/i>, 285<\/b>, 26358\u201326368 (2010).","DOI":"10.1074\/jbc.M110.147439"},{"key":"9","unstructured":"9) Watanabe M, Osada J, Aratani Y, Kluckman K, Reddick R, Malinow MR, Maeda N. Mice deficient in cystathionine beta-synthase: animal models for mild and severe homocyst(e)inemia. Proc. Natl. Acad. Sci. U.S.A.<\/i>, 92<\/b>, 1585\u20131589 (1995)."},{"key":"10","doi-asserted-by":"crossref","unstructured":"10) Yamamoto J, Kamata S, Miura A, Nagata T, Kainuma R, Ishii I. Differential adaptive responses to 1- or 2-day fasting in various mouse tissues revealed by quantitative PCR analysis. FEBS Open Bio.<\/i>, 5<\/b>, 357\u2013368 (2015).","DOI":"10.1016\/j.fob.2015.04.012"},{"key":"11","doi-asserted-by":"crossref","unstructured":"11) Akahoshi N, Kamata S, Kubota M, Hishiki T, Nagahata Y, Matsuura T, Yamazaki C, Yoshida Y, Yamada H, Ishizaki Y, Suematsu M, Kasahara T, Ishii I. Neutral aminoaciduria in cystathionine beta-synthase-deficient mice, an animal model of homocystinuria. Am. J. Physiol. Renal Physiol.<\/i>, 306<\/b>, F1462\u2013F1476 (2014).","DOI":"10.1152\/ajprenal.00623.2013"},{"key":"12","doi-asserted-by":"crossref","unstructured":"12) Kamata S, Yamamoto J, Ohtani H, Tosaka Y, Yoshikawa S, Akahoshi N, Ishii I. 2D DIGE proteomic analysis reveals fasting-induced protein remodeling through organ-specific transcription factor(s) in mice. FEBS Open Bio.<\/i>, 8<\/b>, 1524\u20131543 (2018).","DOI":"10.1002\/2211-5463.12497"},{"key":"13","unstructured":"13) Yang G, Wu L, Jiang B, Yang W, Qi J, Cao K, Meng Q, Mustafa AK, Mu W, Zhang S, Snyder SH, Wang R. H2S as a physiologic vasorelaxant: hypertension in mice with deletion of cystathionine gamma-lyase. Science<\/i>, 322<\/b>, 587\u2013590 (2008)."},{"key":"14","doi-asserted-by":"crossref","unstructured":"14) Yamada H, Akahoshi N, Kamata S, Hagiya Y, Hishiki T, Nagahata Y, Matsuura T, Takano N, Mori M, Ishizaki Y, Izumi T, Kumagai Y, Kasahara T, Suematsu M, Ishii I. Methionine excess in diet induces acute lethal hepatitis in mice lacking cystathionine gamma-lyase, an animal model of cystathioninuria. Free Radic. Biol. Med.<\/i>, 52<\/b>, 1716\u20131726 (2012).","DOI":"10.1016\/j.freeradbiomed.2012.02.033"},{"key":"15","doi-asserted-by":"crossref","unstructured":"15) Hagiya Y, Kamata S, Mitsuoka S, Okada N, Yoshida S, Yamamoto J, Ohkubo R, Abiko Y, Yamada H, Akahoshi N, Kasahara T, Kumagai Y, Ishii I. Hemizygosity of transsulfuration genes confers increased vulnerability against acetaminophen-induced hepatotoxicity in mice. Toxicol. Appl. 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