正常胎儿和杜氏肌营养不良症中的肌肉转酮醇酶

Muscle transketolase in normal foetuses and Duchenne dystrophy.

作者信息

Banerji A P, Karandikar P V, Varadkar A M, Desai A D, Potnis A V

出版信息

Clin Chim Acta. 1978 Jun 15;86(3):307-11. doi: 10.1016/0009-8981(78)90386-8.

Abstract

The activity of the enzyme transketolase (EC 2.2.1.1) or the pentose phosphate metabolizing enzyme (PPME) was investigated in the quadriceps muscles of patients with Duchenne muscular dystrophy (DMD), polymyositis and spinal muscular atrophy (SMA) and in normal foetuses. The enzyme activity was significantly elevated in these muscle disorders with highest and lowest levels seen in DMD and SMA groups, respectively. The enzyme activity was increased early in DMD muscle and appeared to increase with progression of the disease. Normal foetal muscle contained high transketolase activity which is comparable to that seen in DMD patients.

摘要

在杜兴氏肌营养不良症（DMD）、多发性肌炎和脊髓性肌萎缩症（SMA）患者的股四头肌以及正常胎儿中，研究了转酮醇酶（EC 2.2.1.1）或戊糖磷酸代谢酶（PPME）的活性。在这些肌肉疾病中，该酶活性显著升高，分别在DMD组和SMA组中观察到最高和最低水平。在DMD肌肉中，该酶活性在疾病早期就升高，并且似乎随着疾病进展而增加。正常胎儿肌肉含有较高的转酮醇酶活性，这与DMD患者中所见的相当。