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. 2024 May 29;43(14):3009–3026. doi: 10.1038/s44318-024-00128-y

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© The Author(s) 2024

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. Creative Commons Public Domain Dedication waiver http://creativecommons.org/publicdomain/zero/1.0/ applies to the data associated with this article, unless otherwise stated in a credit line to the data, but does not extend to the graphical or creative elements of illustrations, charts, or figures. This waiver removes legal barriers to the re-use and mining of research data. According to standard scholarly practice, it is recommended to provide appropriate citation and attribution whenever technically possible.

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(A) Simplified scheme of the complement cascade activation with highlighted checkpoints controlled by C4BP. (B) Two subunits are forming the C4BP higher-order structures in human serum, namely C4BPα in gray and C4BPβ in blue. Complexing partner ProS is shown in teal. Propeptides and signal peptides are visualized in black. Complement control protein (CCP) domains of C4BPα and C4BPβ are numbered from the N- to C-terminus 1–8 and 1–3, respectively. ProS is composed of the N-terminal gamma-carboxy-glutamate domain (GLA), four EGF-like domains (E1–E4), and two Laminin G-like domains (LG1–2). (C) Cartoon representation of proposed co-occurring isoforms of human serum C4BP: α7β1+ProS, α6β1+ProS, and 7α.