C1-Esterase Inhibitor: An Anti-inflammatory Agent And Its Potential ...

Clipboard, Search History, and several other advanced features are temporarily unavailable. Skip to main page content

The .gov means it’s official. Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

The site is secure. The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation Search: Search Advanced Clipboard Save Email Send to

• Clipboard
• My Bibliography
• Collections
• Citation manager

Display options Display options Format Abstract PubMed PMID

Save citation to file

Format: Summary (text) PubMed PMID Abstract (text) CSV Create file Cancel

Email citation

Subject: 1 selected item: 10699156 - PubMed To: From: Format: Summary Summary (text) Abstract Abstract (text) MeSH and other data Send email Cancel

Add to Collections

• Create a new collection
• Add to an existing collection

Name your collection: Name must be less than 100 characters Choose a collection: Unable to load your collection due to an error Please try again Add Cancel

Add to My Bibliography

• My Bibliography

Unable to load your delegates due to an error Please try again Add Cancel

Your saved search

Name of saved search: Search terms: Test search terms Would you like email updates of new search results? Saved Search Alert Radio Buttons

• Yes
• No

Email: (change) Frequency: Monthly Weekly Daily Which day? The first Sunday The first Monday The first Tuesday The first Wednesday The first Thursday The first Friday The first Saturday The first day The first weekday Which day? Sunday Monday Tuesday Wednesday Thursday Friday Saturday Report format: Summary Summary (text) Abstract Abstract (text) PubMed Send at most: 1 item 5 items 10 items 20 items 50 items 100 items 200 items Send even when there aren't any new results Optional text in email: Save Cancel

Create a file for external citation management software

Create file Cancel

Your RSS Feed

Name of RSS Feed: Number of items displayed: 5 10 15 20 50 100 Create RSS Cancel RSS Link Copy

Full text links

HighWire Full text links

Actions

CiteCollectionsAdd to Collections

• Create a new collection
• Add to an existing collection

Name your collection: Name must be less than 100 characters Choose a collection: Unable to load your collection due to an errorPlease try again Add Cancel Display options Display options Format AbstractPubMedPMID

Share

• Permalink Copy

Page navigation

• Title & authors
• Abstract
• Similar articles
• Cited by
• Publication types
• MeSH terms
• Substances
• Related information
• LinkOut - more resources

Title & authors Abstract Similar articles Cited by Publication types MeSH terms Substances Related information LinkOut - more resources Full text links CiteDisplay options Display options Format AbstractPubMedPMID

Abstract

C1-esterase inhibitor (C1-Inh) therapy was introduced in clinical medicine about 25 years ago as a replacement therapy for patients with hereditary angioedema caused by a deficiency of C1-Inh. There is now accumulating evidence, obtained from studies in animals and observations in patients, that administration of C1-Inh may have a beneficial effect as well in other clinical conditions such as sepsis, cytokine-induced vascular leak syndrome, acute myocardial infarction, or other diseases. Activation of the complement system, the contact activation system, and the coagulation system has been observed in these diseases. A typical feature of the contact and complement system is that on activation they give rise to vasoactive peptides such as bradykinin or the anaphylatoxins, which in part explains the proinflammatory effects of either system. C1-Inh, belonging to the superfamily of serine proteinase inhibitors (serpins), is a major inhibitor of the classical complement pathway, the contact activation system, and the intrinsic pathway of coagulation, respectively. It is, therefore, endowed with anti-inflammatory properties. However, inactivation of C1-Inh occurs locally in inflamed tissues by proteolytic enzymes (e.g., elastase) released from activated neutrophils or bacteria thereby leading to increased local activation of the various host defense systems. Here we will give an overview on the biochemistry and biology of C1-Inh. We will discuss studies addressing therapeutic administration of C1-Inh in experimental and clinical conditions. Finally, we will provide an explanation for the therapeutic benefit of C1-Inh in so many different diseases.

PubMed Disclaimer

Similar articles

• Initial studies on the administration of C1-esterase inhibitor to patients with septic shock or with a vascular leak syndrome induced by interleukin-2 therapy. Hack CE, Ogilvie AC, Eisele B, Jansen PM, Wagstaff J, Thijs LG. Hack CE, et al. Prog Clin Biol Res. 1994;388:335-57. Prog Clin Biol Res. 1994. PMID: 7831367 Review.
• Influence of C1-inhibitor on inflammation, edema and shock. Dickneite G. Dickneite G. Behring Inst Mitt. 1993 Dec;(93):299-305. Behring Inst Mitt. 1993. PMID: 8172580 Review.
• Mechanisms of complement activation and effects of C1-inhibitor on the meconium-induced inflammatory reaction in human cord blood. Salvesen B, Nielsen EW, Harboe M, Saugstad OD, Mollnes TE. Salvesen B, et al. Mol Immunol. 2009 Feb;46(4):688-94. doi: 10.1016/j.molimm.2008.09.001. Epub 2008 Oct 31. Mol Immunol. 2009. PMID: 18950866
• Angioedema due to acquired C1-esterase inhibitor deficiency in a patient with Helicobacter pylori infection. Farkas H, Gyeney L, Majthényi P, Füst G, Varga L. Farkas H, et al. Z Gastroenterol. 1999 Jun;37(6):513-8. Z Gastroenterol. 1999. PMID: 10427658
• Administration of C1-esterase inhibitor during emergency coronary artery bypass surgery in acute ST-elevation myocardial infarction. Thielmann M, Marggraf G, Neuhäuser M, Forkel J, Herold U, Kamler M, Massoudy P, Jakob H. Thielmann M, et al. Eur J Cardiothorac Surg. 2006 Aug;30(2):285-93. doi: 10.1016/j.ejcts.2006.04.022. Epub 2006 Jul 7. Eur J Cardiothorac Surg. 2006. PMID: 16829095 Clinical Trial.

See all similar articles

Cited by

• Prolonging the circulatory half-life of C1 esterase inhibitor via albumin fusion. Sivananthan S, Bhakta V, Chaechi Tehrani N, Sheffield WP. Sivananthan S, et al. PLoS One. 2024 Oct 23;19(10):e0305719. doi: 10.1371/journal.pone.0305719. eCollection 2024. PLoS One. 2024. PMID: 39441778 Free PMC article.
• Adipose tissue derived stem cell secretome induces motor and histological gains after complete spinal cord injury in Xenopus laevis and mice. Assunção-Silva RC, Pinho A, Cibrão JR, Pereira IM, Monteiro S, Silva NA, Campos J, Rebelo AL, Schlosser G, Pinto L, Pandit A, Salgado AJ. Assunção-Silva RC, et al. J Tissue Eng. 2024 Feb 9;15:20417314231203824. doi: 10.1177/20417314231203824. eCollection 2024 Jan-Dec. J Tissue Eng. 2024. PMID: 38343771 Free PMC article.
• Cerebrospinal Fluid C1-Esterase Inhibitor and Tie-1 Levels Affect Cognitive Performance: Evidence from Proteome-Wide Mendelian Randomization. Zagkos L, Dib MJ, Cronjé HT, Elliott P, Dehghan A, Tzoulaki I, Gill D, Daghlas I. Zagkos L, et al. Genes (Basel). 2024 Jan 4;15(1):71. doi: 10.3390/genes15010071. Genes (Basel). 2024. PMID: 38254961 Free PMC article.
• Association of Blood Glucose Variability with Sepsis-Related Disseminated Intravascular Coagulation Morbidity and Mortality. Liu D, Fan Y, Zhuang Y, Peng H, Gao C, Chen Y. Liu D, et al. J Inflamm Res. 2022 Nov 30;15:6505-6516. doi: 10.2147/JIR.S383053. eCollection 2022. J Inflamm Res. 2022. PMID: 36474519 Free PMC article.
• C1-inhibitor influence on platelet activation by thrombin receptors agonists. Tarandovskiy ID, Buehler PW, Karnaukhova E. Tarandovskiy ID, et al. Clin Appl Thromb Hemost. 2022 Jan-Dec;28:10760296221120422. doi: 10.1177/10760296221120422. Clin Appl Thromb Hemost. 2022. PMID: 35996317 Free PMC article.

See all "Cited by" articles

Publication types

• Research Support, Non-U.S. Gov't Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Review Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search

MeSH terms

• Animals Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Anti-Inflammatory Agents, Non-Steroidal / therapeutic use* Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Complement C1 Inactivator Proteins / physiology Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Complement C1 Inactivator Proteins / therapeutic use* Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Complement C1 Inhibitor Protein Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Glycosaminoglycans / metabolism Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Humans Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Inflammation / drug therapy* Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Leukocytes / drug effects Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search

Substances

• Anti-Inflammatory Agents, Non-Steroidal Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Complement C1 Inactivator Proteins Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Complement C1 Inhibitor Protein Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search
• Glycosaminoglycans Actions
  • Search in PubMed
  • Search in MeSH
  • Add to Search

Related information

• MedGen

LinkOut - more resources

• Full Text Sources

  • HighWire

• Other Literature Sources

  • The Lens - Patent Citations Database

Full text links [x] HighWire [x] Cite Copy Download .nbib .nbib Format: AMA APA MLA NLM Send To

• Clipboard
• Email
• Save
• My Bibliography
• Collections
• Citation Manager

[x]

NCBI Literature Resources

MeSH PMC Bookshelf Disclaimer

The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). Unauthorized use of these marks is strictly prohibited.