Clinical and Experimental Pediatrics

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Yoshiyuki Okano (Okano Y)

1 Article

Original Article

Pterin & DHPR Measurement and DNA Analysis in Korean PKU Patients

Chol Hee Chong, Hye Yong Lee, Dong Hwan Lee, Sang Jhoo Lee, Ki Weon Cha, Jeong Bin Yim, Yoshiyuki Okano

Clin Exp Pediatr. 1993;36(12):1681-1690. Published online December 15, 1993

Phenylketonuria in metabolic disorder that results from a deficiency of the hepatic phenylalanine hydroxylase. But among patients with hyperphenylalaninemia, the defect resides in one of the enzymes necessary for production or recycling of tetrahydrobiopterin (BH4). The reduction of BH4 affects not only phenylalanine metabolism but also formation of the tyrosine related neurotransmitter, dopamin and tryptophan related neurotransmitter, serotonin. Administration of L-dopa...

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