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Circulating tricarboxylic acid cycle metabolite levels in citrin-deficient children with metabolic adaptation, with and without sodium pyruvate treatment.
Nagasaka H, Komatsu H, Inui A, Nakacho M, Morioka I, Tsukahara H, Kaji S, Hirayama S, Miida T, Kondou H, Ihara K, Yagi M, Kizaki Z, Bessho K, Kodama T, Iijima K, Saheki T, Yorifuji T, Honda A. Nagasaka H, et al. Among authors: tsukahara h. Mol Genet Metab. 2017 Mar;120(3):207-212. doi: 10.1016/j.ymgme.2016.12.011. Epub 2016 Dec 24. Mol Genet Metab. 2017. PMID: 28041819
Sustaining hypercitrullinemia, hypercholesterolemia and augmented oxidative stress in Japanese children with aspartate/glutamate carrier isoform 2-citrin-deficiency even during the silent period.
Nagasaka H, Okano Y, Tsukahara H, Shigematsu Y, Momoi T, Yorifuji J, Miida T, Ohura T, Kobayashi K, Saheki T, Hirano K, Takayanagi M, Yorifuji T. Nagasaka H, et al. Among authors: tsukahara h. Mol Genet Metab. 2009 May;97(1):21-6. doi: 10.1016/j.ymgme.2009.01.009. Epub 2009 Jan 25. Mol Genet Metab. 2009. PMID: 19232506
Experimental evidence that phenylalanine is strongly associated to oxidative stress in adolescents and adults with phenylketonuria.
Sanayama Y, Nagasaka H, Takayanagi M, Ohura T, Sakamoto O, Ito T, Ishige-Wada M, Usui H, Yoshino M, Ohtake A, Yorifuji T, Tsukahara H, Hirayama S, Miida T, Fukui M, Okano Y. Sanayama Y, et al. Among authors: tsukahara h. Mol Genet Metab. 2011 Jul;103(3):220-5. doi: 10.1016/j.ymgme.2011.03.019. Epub 2011 Mar 29. Mol Genet Metab. 2011. PMID: 21514861
Serum Amino Acid Profiling in Citrin-Deficient Children Exhibiting Normal Liver Function During the Apparently Healthy Period.
Miyazaki T, Nagasaka H, Komatsu H, Inui A, Morioka I, Tsukahara H, Kaji S, Hirayama S, Miida T, Kondou H, Ihara K, Yagi M, Kizaki Z, Bessho K, Kodama T, Iijima K, Yorifuji T, Matsuzaki Y, Honda A. Miyazaki T, et al. Among authors: tsukahara h. JIMD Rep. 2019;43:53-61. doi: 10.1007/8904_2018_99. Epub 2018 Apr 14. JIMD Rep. 2019. PMID: 29654547 Free PMC article.
Cholesterol Metabolism Is Enhanced in the Liver and Brain of Children With Citrin Deficiency.
Hirayama S, Nagasaka H, Honda A, Komatsu H, Kodama T, Inui A, Morioka I, Kaji S, Ueno T, Ihara K, Yagi M, Kizaki Z, Bessho K, Kondou H, Yorifuji T, Tsukahara H, Iijima K, Miida T. Hirayama S, et al. Among authors: tsukahara h. J Clin Endocrinol Metab. 2018 Jul 1;103(7):2488-2497. doi: 10.1210/jc.2017-02664. J Clin Endocrinol Metab. 2018. PMID: 29659898
Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.
Nagasaka H, Yorifuji T, Kobayashi K, Takikawa H, Komatsu H, Inui A, Fujisawa T, Miida T, Tsukahara H, Takatani T, Hayashi H. Nagasaka H, et al. Among authors: tsukahara h. Mol Genet Metab. 2010 Jun;100(2):123-8. doi: 10.1016/j.ymgme.2010.02.008. Epub 2010 Feb 16. Mol Genet Metab. 2010. PMID: 20219403
Fatty liver and anti-oxidant enzyme activities along with peroxisome proliferator-activated receptors γ and α expressions in the liver of Wilson's disease.
Nagasaka H, Miida T, Inui A, Inoue I, Tsukahara H, Komatsu H, Hiejima E, Fujisawa T, Yorifuji T, Hiranao K, Okajima H, Inomata Y. Nagasaka H, et al. Among authors: tsukahara h. Mol Genet Metab. 2012 Nov;107(3):542-7. doi: 10.1016/j.ymgme.2012.08.004. Epub 2012 Aug 11. Mol Genet Metab. 2012. PMID: 22940187
Growth hormone activates hepatic and cerebral cholesterol metabolism in small-for-gestational age children without catch-up growth.
Hirayama S, Nagasaka H, Nakagawa S, Takuwa M, Nakacho M, Yorifuji T, Kondou H, Tsukahara H, Morioka I, Ishida A, Yamato S, Miida T. Hirayama S, et al. Among authors: tsukahara h. J Clin Lipidol. 2017 Jul-Aug;11(4):1032-1042. doi: 10.1016/j.jacl.2017.05.010. Epub 2017 Jun 3. J Clin Lipidol. 2017. PMID: 28826566 Clinical Trial.
345 results