TY - CHAP M1 - Book, Section TI - Hyperphenylalaninemias: Disorders of Tetrahydrobiopterin Metabolism A1 - Blau, Nenad A1 - Thöny, Beat A2 - Sarafoglou, Kyriakie A2 - Hoffmann, Georg F. A2 - Roth, Karl S. Y1 - 2017 N1 - T2 - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e AB - Tetrahydrobiopterin (BH4) is essential for diverse processes and is ubiquitously present in all tissues of higher organisms. The best investigated functions of BH4 are as a natural cofactor of the following eight enzymes:1 phenylalanine-4-hydroxylase (PAH), tyrosine-3-hydroxylase (TH), tryptophan-5-hydroxylase (TPH1 and TPH2), nitric oxide synthase (nNOS, iNOS, and eNOS), and glyceryl-ether monooxygenase (GEMO). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/23 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1140316581 ER -