title: An extracorporeal hollow-fiber reactor for phenylketonuria using immobilized phenylalanine ammonia lyase.
abstract: A hollow-fiber hemodialyzer with immobilized phenylalanine ammonia lyase was tested in vitro for depletion of blood phenylalanine in a recirculating system. A sustained reduction of phenylalanine was obtained in less than 1 h. The product of phenylalanine deamination, trans-cinnamic acid, is a nontoxic compound metabolized to benzoic acid by the liver and eliminated in the urine as hippuric acid. As a model, this reactor may be relevant not only for the short-term management of hyperphenylalaninemia (particularly in pregnant phenylketonuric mothers), but for other metabolic diseases as well, provided that a biocatalyst effective on the accumulating substance is available.
mesh_terms: D000642:Ammonia-Lyases; D004800:Enzymes, Immobilized; D005112:Extracorporeal Circulation / Q000295:instrumentation*; D006801:Humans; D010649:Phenylalanine / Q000097:blood; D010650:Phenylalanine Ammonia-Lyase; D010661:Phenylketonurias / Q000097:blood / Q000628:therapy*; D006435:Renal Dialysis; D013997:Time Factors
chemical_list: D004800:Enzymes, Immobilized; D010649:Phenylalanine; D000642:Ammonia-Lyases; D010650:Phenylalanine Ammonia-Lyase