Complement C1 Inhibitor Protein (C1 Esterase Inhibitor)

An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.

Also Known As:

C1 Esterase Inhibitor; C1-INH Protein; C1-Inhibitor Protein; Complement C1-Inhibitor Protein; Plasma Protease C1 Inhibitor; SERPING1; Serpin Family G Member 1; Serpin G1; C1 INH Protein; C1 Inhibitor Protein; Esterase Inhibitor, C1

Networked: 502 relevant articles (21 outcomes, 43 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Bernstein, Jonathan A: 22 articles (01/2022 - 10/2009)
2.	Farkas, Henriette: 16 articles (01/2022 - 12/2003)
3.	Cicardi, Marco: 16 articles (04/2021 - 09/2004)
4.	Craig, Timothy J: 14 articles (05/2015 - 10/2008)
5.	Relan, Anurag: 12 articles (04/2021 - 01/2012)
6.	Grivcheva-Panovska, Vesna: 8 articles (04/2021 - 10/2009)
7.	Bork, Konrad: 8 articles (03/2020 - 05/2003)
8.	Moldovan, Dumitru: 8 articles (11/2019 - 10/2009)
9.	Riedl, Marc A: 8 articles (02/2019 - 01/2012)
10.	López-Lera, Alberto: 7 articles (08/2021 - 10/2011)

Related Diseases

1.	Hereditary Angioedemas 10/01/2009 - "C1 esterase inhibitor concentrate given intravenously at a dose of 20 U/kg is an effective and safe treatment for acute abdominal and facial attacks in patients with hereditary angioedema, with a rapid onset of relief." 01/01/2011 - "One such product, C1 esterase inhibitor, has now been shown in a rigorous double-blind study to be effective in prevention of hereditary angioedema attacks and has been approved by the US Federal Drug Administration for prophylaxis of the disease. " 05/01/2015 - "Although treatment with C1 esterase inhibitor (C1-INH) concentrate is well established for hereditary angioedema (HAE) attacks in general, data that assess its efficacy for cutaneous attack treatment are sparse. " 01/01/2021 - "Trial registration A Study to Evaluate the Long-term Clinical Safety and Efficacy of Subcutaneously Administered C1-esterase Inhibitor in the Prevention of Hereditary Angioedema, NCT02316353. " 09/28/2018 - "Efficacy of recombinant human C1 esterase inhibitor across anatomic locations in acute hereditary angioedema attacks."
2.	Angioedema 01/01/2022 - "Pediatric patients suspected to have HAE based on the presence of angioedema and screened for C1 esterase inhibitor levels and/or function were included in the study. " 11/01/2021 - "The purpose of this study was to evaluate the use, clinical efficacy, and angioedema-related medication costs of C1 esterase inhibitor (C1EI) for ACEi-induced angioedema. " 12/01/2022 - "Recombinant human C1 esterase inhibitor as prophylactic treatment in idiopathic non-histaminergic angioedema." 04/01/2022 - "The characteristic of HAE is recurrent angioedema episodes due to low C1 esterase inhibitor (C1-INH) level. " 01/01/2022 - "Targeted re-sequencing of five HAE-associated genes (SERPING1, F12, PLG, ANGPT1, and KNG1) was performed in 212 ACEi/ARB-induced angioedema patients recruited in Germany/Austria, Sweden, and Denmark, and in 352 controls from a German cohort. "
3.	Delayed Graft Function 01/01/2020 - "Three-Year Outcomes of a Randomized, Double-Blind, Placebo-Controlled Study Assessing Safety and Efficacy of C1 Esterase Inhibitor for Prevention of Delayed Graft Function in Deceased Donor Kidney Transplant Recipients." 12/01/2018 - "A phase I/II, double-blind, placebo-controlled study assessing safety and efficacy of C1 esterase inhibitor for prevention of delayed graft function in deceased donor kidney transplant recipients." 01/01/2020 - "We previously reported from a randomized, placebo-controlled trial that treatment with C1 esterase inhibitor was associated with a shorter duration of delayed graft function and higher eGFR at 1 year. " 01/01/2020 - "Treatment of patients at risk for ischemia-reperfusion injury and delayed graft function with C1 esterase inhibitor was associated with a lower incidence of graft failure."
4.	Reperfusion Injury 02/04/1997 - "The present study addresses the possibility of reducing reperfusion damage by the application of C1 esterase inhibitor (C1-INH). " 03/01/2021 - "Effects of Ischemic Preconditioning and C1 Esterase Inhibitor Administration following Ischemia-Reperfusion Injury in a Rat Skin Flap Model." 01/01/2013 - "We therefore tested preoperative treatment for prevention of reperfusion injury by using C1 esterase inhibitor (C1 INH). " 01/01/2013 - "C1 esterase inhibitor reduces lower extremity ischemia/reperfusion injury and associated lung damage." 02/01/2017 - "C1 esterase inhibitor ameliorates ischemia reperfusion injury in a swine musculocutaneous flap model."
5.	Acute Kidney Injury (Acute Renal Failure) 01/01/2022 - "Tubule-mitophagic secretion of SerpinG1 reprograms macrophages to instruct anti-septic acute kidney injury efficacy of high-dose ascorbate mediated by NRF2 transactivation." 01/01/2020 - "This study sought to determine the efficacy profile and safety of recombinant human C1 esterase inhibitor (rhC1INH) in the prevention of contrast-associated acute kidney injury after elective coronary angiography. " 10/01/1998 - "We studied seven variables of the coagulation pathway (PT, aPTT, fibrinogen, FVIII:c, von Willebrand factor, thrombin-antithrombin complexes, prothrombin fragments 1+2) and seven parameters of the fibrinolytic system (plasminogen, alpha2-antiplasmin, C1-esterase inhibitor, tissue-type plasminogen activator, urokinase-type plasminogen activator, plasminogen activator inhibitor type 1, D-dimer) in 24 pediatric patients with diarrhea-associated hemolytic uremic syndrome and in 15 children with acute renal failure not due to hemolytic uremic syndrome. "

Related Drugs and Biologics

1.	Complement System Proteins (Complement)
2.	icatibant
3.	Angiotensin-Converting Enzyme Inhibitors (ACE Inhibitors)
4.	Antithrombin III
5.	Histamine Antagonists (Antihistamines)
6.	Adrenal Cortex Hormones (Corticosteroids)
7.	Bradykinin B2 Receptor
8.	Fibrin
9.	Omalizumab (Xolair)
10.	anti-IgE antibodies

Related Therapies and Procedures

1.	Therapeutics
2.	Kidney Transplantation
3.	Liver Transplantation
4.	Myocutaneous Flap
5.	Noninvasive Ventilation