Melamed J, Alper C A, Cicardi M, Rosen F S
J Allergy Clin Immunol. 1986 Feb;77(2):322-6. doi: 10.1016/s0091-6749(86)80111-7.
The metabolism of 125I-labeled C1 inhibitor (C1INH) and C1q was studied in five patients with B cell lymphoproliferative disorders, C1INH deficiency, and angioedema. C1INH catabolism was markedly accelerated in these patients. The fractional catabolic rate (FCR) was 0.053 of the plasma pool per hour compared to that of normal subjects (0.025) or patients with hereditary angioneurotic edema (HANE) (0.035). The catabolism of two dysfunctional proteins Wel and Ta was studied. Protein Wel was catabolized at an accelerated rate (0.041) compared to that in patients with HANE (0.029) or in normal subjects (0.020). In contrast, the FCR of protein Ta was 0.012, which is similar to that in normal patients and in patients with HANE. The extravascular to plasma ratio (E/P) of the normal C1INH in patients was 1.55 compared to 0.60 in normal patients. This is consistent with the rapid extravascular sequestration of the C1INH. The synthesis rate of the C1INH was 0.29 mg/kg/hr in patients that is similar to that in control subjects. The metabolism of C1q was studied in two normal control subjects and three patients. The FCR of C1q was 0.051 in patients compared to 0.023 in control subjects. The E/P was increased in patients (2.8) compared to E/P in control subjects (0.6). The acquisition of C1INH deficiency results from increased consumption of C1INH in vivo.
在5例患有B细胞淋巴增生性疾病、C1抑制物（C1INH）缺乏症和血管性水肿的患者中，对125I标记的C1INH和C1q的代谢进行了研究。这些患者的C1INH分解代谢明显加快。与正常受试者（0.025）或遗传性血管性水肿（HANE）患者（0.035）相比，血浆池的分解代谢率（FCR）为每小时0.053。研究了两种功能失调蛋白Wel和Ta的分解代谢。与HANE患者（0.029）或正常受试者（0.020）相比，蛋白Wel以加速速率（0.041）分解代谢。相比之下，蛋白Ta的FCR为0.012，这与正常患者和HANE患者相似。患者中正常C1INH的血管外与血浆比率（E/P）为1.55，而正常患者为0.60。这与C1INH快速的血管外隔离一致。患者中C1INH的合成速率为0.29mg/kg/小时，与对照受试者相似。在2名正常对照受试者和3名患者中研究了C1q的代谢。患者中C1q的FCR为0.051，而对照受试者为0.023。与对照受试者的E/P（0.6）相比，患者的E/P增加（2.8）。C1INH缺乏症的形成是由于体内C1INH消耗增加所致。
