Three RFLPs at the 3' end of the cystatin C gene, the disease gene in hereditary cystatin C amyloid angiopathy (HCCAA) in Iceland.
Three RFLPs at the 3' end of the cystatin C gene, the disease gene in hereditary cystatin C amyloid angiopathy (HCCAA) in Iceland.
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胱抑素 C 基因 3 端有 3 个 RFLP，该基因是冰岛遗传性胱抑素 C 淀粉样血管病 (HCCAA) 的疾病基因。
DOI：
10.1093/nar/18.24.7471-a
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发表时间：
1990
影响因子：
14.9
通讯作者：
Ó. Jensson
中科院分区：
文献类型：
--
作者：
A. Palsdottir;S. Jónsdóttir;M. Abrahamson;A. Grubb;Ó. Jensson
对 25 名支气管扩张患者的痰样本进行了酶法测定髓过氧化物酶、中性粒细胞弹性蛋白酶和组织蛋白酶 B，并进行免疫学测定胱抑素 A、胱抑素 B、胱抑素 C、胱抑素 S 和激肽原。在那些目视评估为脓性的痰样本中发现了高髓过氧化物酶和中性粒细胞弹性蛋白酶水平。这些样本还被发现含有高水平的组织蛋白酶 B 活性和半胱氨酸蛋白酶抑制剂 A，但半胱氨酸蛋白酶抑制剂 S 和最有效的组织蛋白酶 B 抑制剂半胱氨酸蛋白酶抑制剂 C 的水平较低。相反，髓过氧化物酶和中性粒细胞弹性蛋白酶活性较低的痰样本中组织蛋白酶 B 和半胱氨酸蛋白酶抑制剂 A 的水平较低，但半胱氨酸蛋白酶抑制剂 C 和 S 的水平较高。结论是痰中的组织蛋白酶B活性与炎症程度和中性粒细胞募集呈正相关。尽管这可能部分是由于组织蛋白酶 B 抑制剂（尤其是半胱氨酸蛋白酶抑制剂 C）的量减少，但理论上的考虑表明，除了抑制剂的总水平之外，还必须有其他因素参与组织蛋白酶 B 活性的控制。 （较少的）
Sputum samples from 25 patients with bronchiectasis were assayed enzymatically for myeloperoxidase, neutrophil elastase and cathepsin B, and immunologically for cystatin A, cystatin B, cystatin C, cystatin S and kininogen. High myeloperoxidase and neutrophil elastase levels were found in those sputum samples that were assessed visually to be purulent. These samples were also found to contain high levels of cathepsin B activity and cystatin A, but low levels of cystatin S and of the most effective cathepsin B inhibitor, cystatin C. In contrast, sputum samples that were low in myeloperoxidase and neutrophil elastase activities had low levels of cathepsin B and cystatin A, but high cystatin C and S levels. It is concluded that cathepsin B activity in sputum is positively correlated with the degree of inflammation and neutrophil recruitment. Although this may be due in part to reduced amounts of cathepsin B inhibitors, particularly cystatin C, theoretical considerations suggest that factors other than the gross level of inhibitors must be involved in the control of cathepsin B activity. (Less)