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- W2079496726 abstract "Multitubular enzyme reactors with immobilized enzymes were developed to achieve depletion of circulating substrate by extracorporeal means. To act as prototypes, reactors were prepared with immobilized L-phenylalanine ammonia-lyase, an enzyme that metabolizes phenylalanine to trans-cinnamic acid and ammonia without the need for a coenzyme. We report the first application of phenylalanine ammonia-lyase reactors in an extracorporeal circulation system in a patient with phenylketonuria. A phenylalanine level of 1.82 mmol/L (for the last 6 years) decreased to 1.24 mmol/L after 5.5. hours of treatment, without the enzyme entering the circulation. Total phenylalanine depletion from blood and tissue stores was estimated at 1800 mg. The hemodialysis-like procedure proved to be without side effects, specific for phenylalanine, and suitable in the management of pregnant women with phenylketonuria and late-onset hyperphenylalaninemia. The extracorporeal use of enzyme reactors for temporary enzyme replacement represents a new, safe, and effective therapeutic modality." @default.
- W2079496726 created "2016-06-24" @default.
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- W2079496726 date "1987-04-01" @default.
- W2079496726 modified "2023-10-14" @default.
- W2079496726 title "Extracorporeal Enzyme Reactors for Depletion of Phenylalanine in Phenylketonuria" @default.
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- W2079496726 doi "https://doi.org/10.7326/0003-4819-106-4-531" @default.
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