Shimizu Y, Kondo T, Kuchiba K, Urata G
J Lab Clin Med. 1977 Mar;89(3):517-23.
The activities of uroporphyrinogen III cosynthetase in blood lysates from five patients with the Dubin-Johnson syndrome (DJS) and four control subjects and in liver homogenates from four patients and four control subjects were determined. No significant difference was found in enzyme activity between the two groups in either blood lysate or liver homogenate. These results indicate that low urinary coproporphyrin III output in the DJS is not due to deficiency of uroporphyrinogen III cosynthetase in the liver and the erythropoietic system.
测定了5例杜宾-约翰逊综合征(DJS)患者和4例对照者血液裂解物中尿卟啉原III合酶的活性,以及4例患者和4例对照者肝脏匀浆中该酶的活性。在血液裂解物或肝脏匀浆中,两组之间的酶活性均未发现显著差异。这些结果表明,DJS患者尿中粪卟啉原III排出量低并非由于肝脏和红细胞生成系统中尿卟啉原III合酶缺乏所致。