PMID: 11916313Mar 28, 2002Paper

Modelling the phenylalanine blood level response during treatment of phenylketonuria

Journal of Inherited Metabolic Disease
U LangenbeckV Hönig

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.

References

Nov 1, 1979·The British Journal of Nutrition·M EliaR Smith
Jan 1, 1992·Journal of Inherited Metabolic Disease·U LangenbeckA Mench-Hoinowski
Sep 1, 1990·Pediatric Research·G N ThompsonD Halliday
Apr 1, 1982·American Journal of Medical Genetics·A E FiedlerS D Cederbaum
Jul 1, 1996·European Journal of Pediatrics·U Wendel, U Langenbeck
Jan 1, 1996·Journal of Inherited Metabolic Disease·E TreacyR G Cotton
Feb 7, 1998·Proceedings of the National Academy of Sciences of the United States of America·Y BoirieB Beaufrère
Jun 24, 1998·The Journal of Clinical Investigation·F J van SpronsenH S Heymans
Mar 17, 1999·Proceedings of the National Academy of Sciences of the United States of America·S Kaufman
Jul 3, 1999·Trends in Genetics : TIG·C R Scriver, P J Waters

❮ Previous
Next ❯

Citations

Jan 23, 2008·Journal of Inherited Metabolic Disease·U Langenbeck
Jul 18, 2009·Journal of Inherited Metabolic Disease·U LangenbeckUNKNOWN German Collaborative Study on Phenylketonuria (PKU) / Hyperphenylalaninemia (HPA)
Apr 9, 2011·Annals of Nutrition & Metabolism·Margreet van RijnFrancjan J van Spronsen

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.