C1 inhibitor deficiency: consensus document

MM Gompels, RJ Lock, M Abinun… - Clinical & …, 2005 - academic.oup.com
We present a consensus document on the diagnosis and management of C1 inhibitor
deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In …

Glycosylation and the complement system

GE Ritchie, BE Moffatt, RB Sim, BP Morgan… - Chemical …, 2002 - ACS Publications
The human complement pathway is a highly efficient recognition and effector system that is
designed to destroy infectious microbes and damaged host material. Over 30 plasma …

Glyco-engineered CHO cell lines producing alpha-1-antitrypsin and C1 esterase inhibitor with fully humanized N-glycosylation profiles

T Amann, AH Hansen, S Kol, HG Hansen… - Metabolic …, 2019 - Elsevier
Recombinant Chinese hamster ovary (CHO) cells are able to provide biopharmaceuticals
that are essentially free of human viruses and have N-glycosylation profiles similar, but not …

Engineering of routes to heparin and related polysaccharides

U Bhaskar, E Sterner, AM Hickey, A Onishi… - Applied microbiology …, 2012 - Springer
Anticoagulant heparin has been shown to possess important biological functions that vary
according to its fine structure. Variability within heparin's structure occurs owing to its …

N-and O-glycans of recombinant human C1 inhibitor expressed in the milk of transgenic rabbits

K Koles, PHC van Berkel, FR Pieper, JH Nuijens… - …, 2004 - academic.oup.com
Abstract Human C1 inhibitor (hC1INH) is a therapeutic N, O-glycoprotein with a growing
number of clinical applications, but the current natural supplies are not likely to meet the …

C1, MBL–MASPs and C1-inhibitor: novel approaches for targeting complement-mediated inflammation

L Beinrohr, J Dobó, P Závodszky, P Gál - Trends in Molecular Medicine, 2008 - cell.com
Complement activation is initiated by the pattern-recognition molecules complement
component C1q, mannose-binding lectin (MBL) and ficolins (H-, L-, M-ficolin), which typically …

C1-inhibitor: structure, functional diversity and therapeutic development

E Karnaukhova - Current Medicinal Chemistry, 2022 - ingentaconnect.com
Human C1-Inhibitor (C1INH), also known as C1-esterase inhibitor, is an important
multifunctional plasma glycoprotein that is uniquely involved in a regulatory network of …

Functional characterization of the recombinant human C1 inhibitor serpin domain: insights into heparin binding

V Rossi, I Bally, S Ancelet, Y Xu… - The Journal of …, 2010 - journals.aai.org
Variants of the human C1 inhibitor serpin domain containing three N-linked carbohydrates at
positions 216, 231, and 330 (C1inhΔ97), a single carbohydrate at position 330 …

Serpins and the complement system

L Beinrohr, TA Murray-Rust, L Dyksterhuis… - Methods in …, 2011 - Elsevier
Abstract C1-inhibitor (serpin G1) is a 105 kDa inhibitor which functions as a major
antiinflammatory protein in the body. It has its effects via inhibition of the proteases of the …

Obstetrical complications and outcome in two families with hereditary angioedema due to mutation in the F12 gene

O Picone, AC Donnadieu, FG Brivet… - Obstetrics and …, 2010 - Wiley Online Library
Backgroud. Hereditary angioedema (HAE) is characterized by recurrent swelling of the skin,
the abdomen (causing severe acute pain), and the airways. A recently discovered type …