Sissons J G, Liebowitch J, Amos N, Peters D K
J Clin Invest. 1977 Apr;59(4):704-15. doi: 10.1172/JCI108689.
The metabolism of the fifth component of complement (C5), and its relatonship to metabolism of the third component of complement (C3), has been studied in normal subjects and patients by simultaneous administration of radioiodine labeled C5 and C3. In seven normal subjects the fractional catabolic rate of C5 ranged from 1.5 to 2.1% of the plasma pool/h and extravascular/intravascular distribution ratio from 0.22 to 0.78, these values being similar to those obtained for C3, and synthesis rate from 71 to 134 mug/kg per h, In patients with complement activation the increase in fractional catabolic rate of C5 was nearly always less than that of C3. The data also showed that there was increased extravascular distribution of C3 and C5 in most patients and considerable extravascular catabolism of both proteins in some. However, there were differences in metabolic parameters between patients with different types of complement activation. In patients with systemic lupus erythematosus, fractional catabolism and extravascular distribution of C3 and C5 were both increased, and there was marked extravascular catabolism of both proteins. There was increased fractional catabolism and extravascular distribution of C3 in patients with mesangiocapillary nephritis and (or) partial lipodystrophy, and fractional catabolism of C5 was also increased in three of six studies although distribution of C5 was always within the normal range; however, in two patients with nephritic factor in their serum fractional catabolism of C5 was normal despite markedly increased C3 turnover, suggesting that in patients with alternative pathway activation by nephritic factor little or no C5 convertase is generated.
通过同时给予放射性碘标记的补体第五成分(C5)和补体第三成分(C3),对正常受试者和患者体内C5的代谢及其与C3代谢的关系进行了研究。在7名正常受试者中,C5的分解代谢率为血浆池的1.5%至2.1%/小时,血管外/血管内分布率为0.22至0.78,这些值与C3的相似,合成率为71至134微克/千克/小时。在补体激活的患者中,C5分解代谢率的增加几乎总是小于C3。数据还显示,大多数患者的C3和C5血管外分布增加,部分患者两种蛋白质的血管外分解代谢相当可观。然而,不同类型补体激活的患者在代谢参数上存在差异。在系统性红斑狼疮患者中,C3和C5的分解代谢和血管外分布均增加,两种蛋白质均有明显的血管外分解代谢。在系膜毛细血管性肾炎和(或)部分脂肪营养不良患者中,C3的分解代谢和血管外分布增加,在六项研究中的三项中C5的分解代谢也增加,尽管C5的分布始终在正常范围内;然而,在两名血清中有肾炎因子的患者中,尽管C3周转率明显增加,但C5的分解代谢正常,这表明在由肾炎因子激活替代途径的患者中,几乎不产生或不产生C5转化酶。