Phenylalanine depletion for the management of phenylketonuria: use of enzyme reactors with immobilized enzymes.
Multitubular enzyme reactors with immobilized phenylalanine ammonia lyase were tested in vitro and in vivo for depletion of phenylalanine in circulating blood. Sustained reduction of phenylalanine was achieved in less than 30 minutes. A 50% decrease of phenylalanine was obtained with a 2-hour application of enzyme reactors and was maintained for more than 2 days. Similar enzyme reactors have therapeutic potential for temporary management of phenylketonuric patients when their circulating phenylalanine becomes exceedingly high because of infection, fever, or pregnancy.
D000642:Ammonia-Lyases / Q000627:therapeutic use*; D000818:Animals; D004195:Disease Models, Animal; D004285:Dogs; D004800:Enzymes, Immobilized / Q000627:therapeutic use*; D005112:Extracorporeal Circulation; D006801:Humans; D010649:Phenylalanine / Q000097:blood; D010650:Phenylalanine Ammonia-Lyase / Q000627:therapeutic use*; D010661:Phenylketonurias / Q000628:therapy*
D004800:Enzymes, Immobilized; D010649:Phenylalanine; D000642:Ammonia-Lyases; D010650:Phenylalanine Ammonia-Lyase