AA Metabolism IV: Phe and Tyr
Last Modified: 2011-07-16
Related Textbooks:BRS Biochemistry, Molecular Biology, and Genetics, Fifth Edition (Board Review Series)
Related Textbooks:High-Yield Biostatistics (High-Yield Series)
Related Textbooks:Lippincott's Illustrated Reviews: Biochemistry, North American Edition (Lippincott's Illustrated Reviews Series)
Related Textbooks:Medical Genetics: With STUDENT CONSULT Online Access (MEDICAL GENETICS ( JORDE))
Related Textbooks:Robbins Basic Pathology: With STUDENT CONSULT Online Access (Robbins Pathology)
- via transamination to oxaloacetate
- in Urea cycle
phe + O2 + BH4 -> tyr + H2O + BH2
presence of succinylacetone in urine
elevated tyr and phe in blood
- classical PKU - deficiency in phenylalanine hydroxylase
- non-classical PKU - deficiency in BH4 synthesis
excess phenylalanine inhibits uptake of other essential AA
Oral administration of KUVAN (BH4)
Administer l-dopa and 5-hydroxytryptophan
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