title: Modelling the phenylalanine blood level response during treatment of phenylketonuria.
abstract: A vast body of phenylketonuria (PKU) patient monitoring data is deposited in clinical files and, after having served the actual needs, has remained there largely unused. We propose a kinetic model that will allow continued analysis of such data for further elucidation of the patient's metabolic phenotype and phenylalanine (Phe) disposal characteristics. Our PKU model of a single compartment with the input of alimentary Phe and two outputs--(1) first-order Phe conversion to tyrosine and acidic metabolites, and (2) zero-order Phe usage for net protein synthesis--has been developed with the graphics-oriented ModelMaker (then Cherwell Scientific Ltd, Oxford, UK) software package. The corresponding differential and integrated rate equations are presented to enable transfer of the model to equation-oriented simulation packages. The model offers a possible explanation for discrepancies in some genotype-phenotype data.
authors.affiliation: Institute of Human Genetics, University Hospital, Frankfurt/Main, Germany. u.langenbeck@em.uni-frankfurt.de
authors.forename: U
authors.initials: U
authors.lastname: Langenbeck
authors.forename: J
authors.initials: J
authors.lastname: Zschocke
authors.forename: U
authors.initials: U
authors.lastname: Wendel
authors.forename: V
authors.initials: V
authors.lastname: Hönig
mesh_terms: D002648:Child; D002675:Child, Preschool; D003198:Computer Simulation; D005260:Female; D006801:Humans; D007700:Kinetics; D008297:Male; D008954:Models, Biological; D010641:Phenotype; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000097:blood* / Q000145:classification / Q000178:diet therapy*; D014176:Protein Biosynthesis; D012984:Software; D014443:Tyrosine / Q000378:metabolism
chemical_list: D014443:Tyrosine; D010649:Phenylalanine