用于苯丙酮尿症管理的苯丙氨酸清除：固定化酶在酶反应器中的应用

Phenylalanine depletion for the management of phenylketonuria: use of enzyme reactors with immobilized enzymes.

作者信息

Ambrus C M, Ambrus J L, Horvath C, Pedersen H, Sharma S, Kant C, Mirand E, Guthrie R, Paul T

出版信息

Science. 1978 Sep 1;201(4358):837-9. doi: 10.1126/science.567372.

Abstract

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.

摘要

对带有固定化苯丙氨酸解氨酶的多管酶反应器进行了体外和体内测试，以降低循环血液中的苯丙氨酸含量。在不到30分钟内实现了苯丙氨酸的持续减少。使用酶反应器2小时可使苯丙氨酸降低50%，并能维持2天以上。当苯丙酮尿症患者因感染、发烧或怀孕导致循环中的苯丙氨酸含量极高时，类似的酶反应器在临时治疗管理方面具有潜力。