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Auxiliary activation of the complement system and its importance for the pathophysiology of clinical conditions

Huber-Lang, Markus (author)
Univ Hosp Ulm, Germany,University Hospital Ulm, Institute of Clinical and Experimental Trauma-Immunology, Ulm, Germany
Nilsson Ekdahl, Kristina (author)
Uppsala universitet,Linnéuniversitetet,Institutionen för kemi och biomedicin (KOB),Uppsala University, Sweden,Linnaeus Ctr Biomat Chem, BMC,Klinisk immunologi,Linnæus University, Linnæus Center of Biomaterials Chemistry, Kalmar, Sweden
Wiegner, Rebecca (author)
Univ Hosp Ulm, Germany,University Hospital Ulm, Institute of Clinical and Experimental Trauma-Immunology, Ulm, Germany
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Fromell, Karin (author)
Uppsala universitet,Klinisk immunologi,Uppsala University, Sweden
Nilsson, Bo (author)
Uppsala universitet,Klinisk immunologi,Uppsala University, Sweden
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 (creator_code:org_t)
2017-09-12
2018
English.
In: Seminars in Immunopathology. - : Springer. - 1863-2297 .- 1863-2300. ; 40:1, s. 87-102
  • Research review (peer-reviewed)
Abstract Subject headings
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  • 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.

Subject headings

NATURVETENSKAP  -- Biologi -- Immunologi (hsv//swe)
NATURAL SCIENCES  -- Biological Sciences -- Immunology (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP  -- Medicinska och farmaceutiska grundvetenskaper -- Immunologi inom det medicinska området (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Basic Medicine -- Immunology in the medical area (hsv//eng)

Keyword

Complement system
Proteases
Protease inhibitors
Trauma
Immunologi
Immunology

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