Feldman D S, Levere R D, Lieberman J S, Cardinal R A, Watson C J
Proc Natl Acad Sci U S A. 1971 Feb;68(2):383-6. doi: 10.1073/pnas.68.2.383.
The rat hemidiaphragm was studied in vitro as a test system to evaluate the effects of heme precursors and the uroporphyrins upon neuromuscular excitability. Porphobilinogen and porphobilin had no effect on the resting miniature end-plate potential frequency, but the K(+)-augmented frequency was significantly reduced. Porphobilin and porphobilinogen gave 50% of their maximal effect at concentrations of 0.008 and 0.6 mug/ml, respectively; the effect increased with concentration. Uroporphyrin I at 0.05-1.0 mug/ml caused a 25% decrease in frequency, but the effect did not increase with concentration. At similar concentrations, uroporphyrin III was without effect. The concentrations of porphobilin and porphobilinogen effective in inhibiting the K(+) stimulation, which are several orders of magnitude lower than the effective concentrations of simple amino acids, are those which might reasonably be expected in the sera of patients with acute intermittent porphyria.
将大鼠半膈作为体外测试系统,以评估血红素前体和尿卟啉对神经肌肉兴奋性的影响。胆色素原和胆色素对静息微终板电位频率无影响,但钾离子增强的频率显著降低。胆色素和胆色素原分别在浓度为0.008和0.6微克/毫升时产生其最大效应的50%;效应随浓度增加。0.05-1.0微克/毫升的尿卟啉I导致频率降低25%,但效应不随浓度增加。在相似浓度下,尿卟啉III无作用。有效抑制钾离子刺激的胆色素和胆色素原浓度比简单氨基酸的有效浓度低几个数量级,这些浓度在急性间歇性卟啉症患者的血清中可能是合理预期的。