Menegatti E, Bortolotti F, Guarneri M, Scatturin A
Farmaco Sci. 1983 Jun;38(6):397-405.
Ornithine transcarbamylase (OCT) is one of the urea-cycle enzymes in mitochondria which is involved in metabolic disorders in man. Reye's syndrome, characterized by encephalopathy and fatty infiltration of the liver, is one of such diseases. During this syndrome a reduction of OCT specific activity was observed, probably due to increased proteolytic degradation or to alteration in the enzyme conformation. It has recently been found that OCT administration to patients with liver disorders, contributes to illness resolution. Since the therapeutic properties of the enzymes are usually related to their structure, this paper reports some findings obtained by spectroscopic measurements on OCT structure and conformation stability in solution.
鸟氨酸转氨甲酰酶(OCT)是线粒体中的尿素循环酶之一,与人的代谢紊乱有关。瑞氏综合征以脑病和肝脏脂肪浸润为特征,是这类疾病之一。在该综合征期间,观察到OCT比活性降低,这可能是由于蛋白水解降解增加或酶构象改变所致。最近发现,给肝脏疾病患者施用OCT有助于疾病的缓解。由于酶的治疗特性通常与其结构有关,本文报道了通过光谱测量获得的关于溶液中OCT结构和构象稳定性的一些发现。