Depletion of phenylalanine in the blood of phenylketonuric patients using a PAL-enzyme reactor. An in vitro study.
Blood samples obtained from eight adult phenylketonuric individuals had a mean phenylalanine level of 25 mg/dl. When these samples were circulated through multitubular enzyme-reactors prepared with immobilized phenylalanine ammonia lyase an average of 77% of phenylalanine was metabolized within 30 minutes. We conclude that phenylalanine in human phenylketonuric blood is just as susceptible to metabolism by PAL-enzyme reactors as phenylalanine that is added to normal blood, or that is circulating in dogs and monkeys made hyperphenylalaninemic by experimental means.
D000328:Adult; D000642:Ammonia-Lyases / Q000627:therapeutic use*; D004800:Enzymes, Immobilized / Q000627:therapeutic use*; D006801:Humans; D010649:Phenylalanine / Q000097:blood*; D010650:Phenylalanine Ammonia-Lyase / Q000627:therapeutic use*; D010661:Phenylketonurias / Q000097:blood* / Q000628:therapy; D013997:Time Factors
D004800:Enzymes, Immobilized; D010649:Phenylalanine; D000642:Ammonia-Lyases; D010650:Phenylalanine Ammonia-Lyase