Auxiliary activation of the complement system and its importance for the pathophysiology of clinical conditions.
Auxiliary activation of the complement system and its importance for the pathophysiology of clinical conditions.
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DOI：
10.1007/s00281-017-0646-9
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发表时间：
2018-01
影响因子：
9
通讯作者：
Nilsson B
中科院分区：
文献类型：
--
作者：
Huber-Lang M;Ekdahl KN;Wiegner R;Fromell K;Nilsson B
血液的级联系统(补体系统、凝血/接触激活/激肽释放酶系统和纤溶系统)的激活和调节是通过酶原分子激活特定的活性蛋白水解酶来实现的。尽管产生的蛋白酶都存在于血液中，但在生理条件下，产生的蛋白酶的活性受内源性蛋白酶抑制物的控制。因此，流体相中不同系统之间的串扰非常小。这篇概念回顾文章旨在识别和描述规避与系统相关的严格控制的条件。这包括从组织中释放大量蛋白水解酶的临床环境，例如，在胰腺炎或创伤后组织损伤期间，导致特定蛋白酶的天然底物和可用的蛋白酶抑制剂的消耗。级联系统失调的另一个例子是弥漫性血管内凝血，所有血液级联系统的典型激活也导致特定底物和蛋白水解酶抑制物的消除。本综述解释了蛋白酶生物化学的基本概念，对于了解广泛激活的蛋白酶对了解临床情况具有重要意义。
Activation and regulation of the cascade systems of the blood (the complement system, the coagulation/contact activation/kallikrein system, and the fibrinolytic system) occurs via activation of zymogen molecules to specific active proteolytic enzymes. Despite the fact that the generated proteases are all present together in the blood, under physiological conditions, the activity of the generated proteases is controlled by endogenous protease inhibitors. Consequently, there is remarkable little crosstalk between the different systems in the fluid phase. This concept review article aims at identifying and describing conditions where the strict system-related control is circumvented. These include clinical settings where massive amounts of proteolytic enzymes are released from tissues, e.g., during pancreatitis or post-traumatic tissue damage, resulting in consumption of the natural substrates of the specific proteases and the available protease inhibitor. Another example of cascade system dysregulation is disseminated intravascular coagulation, with canonical activation of all cascade systems of the blood, also leading to specific substrate and protease inhibitor elimination. The present review explains basic concepts in protease biochemistry of importance to understand clinical conditions with extensive protease activation.
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DOI：
10.4049/jimmunol.0903678
发表时间：
2010-11-01
期刊：
Journal of immunology (Baltimore, Md. : 1950)
影响因子：
--
作者：
Amara U;Flierl MA;Rittirsch D;Klos A;Chen H;Acker B;Brückner UB;Nilsson B;Gebhard F;Lambris JD;Huber-Lang M
通讯作者：
Huber-Lang M
DOI：
10.2183/pjab.86.524
发表时间：
2010
期刊：
Proceedings of the Japan Academy. Series B, Physical and biological sciences
影响因子：
--
作者：
Amagai M
通讯作者：
Amagai M
影响因子：
2.9
作者：
BOLOTIN, C;MORRIS, S;PRAHL, J
通讯作者：
PRAHL, J
高血清胰蛋白酶水平和 PRSS1 基因的-409 T/T 基因型易患新生儿败血症
影响因子：
5.1
作者：
Chen, Qingquan;Xue, Heng;Chen, Hong
通讯作者：
Chen, Hong
影响因子：
3.6
作者：
EGGERTSEN, G;HELLMAN, U;SJOQUIST, J
通讯作者：
SJOQUIST, J