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The Factor H protein family: The switchers of the complement alternative pathway

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Immunological_Reviews-2022-Lucientes?Continente.pdf (4.67 MB)

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2024-01-16

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Philipps-Universität Marburg
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This publication is with permission of the rights owner freely accessible due to an Alliance licence and a national licence (funded by the DFG, German Research Foundation) respectively
The factor H (FH) protein family is emerging as a complex network of proteins controlling the fate of the complement alternative pathway (AP) and dictating susceptibility to a wide range of diseases including infectious, inflammatory, autoimmune, and degenerative diseases and cancer. Composed, in man, of seven highly related proteins, FH, factor H-like 1, and 5 factor H-related proteins, some of the FH family proteins are devoted to down-regulating the AP, while others exert an opposite function by promoting AP activation. Recent findings have provided insights into the molecular mechanisms defining their biological roles and their pathogenicity, illustrating the relevance that the balance between the regulators and the activators within this protein family has in defining the outcome of complement activation on cell surfaces. In this review we will discuss the emerging roles of the factor H protein family, their impact in the complement cascade, and their involvement in the pathogenesis of complement-mediated diseases associated with the AP dysregulation.
The factor H (FH) protein family is emerging as a complex network of proteins controlling the fate of the complement alternative pathway (AP) and dictating susceptibility to a wide range of diseases including infectious, inflammatory, autoimmune, and degenerative diseases and cancer. Composed, in man, of seven highly related proteins, FH, factor H-like 1, and 5 factor H-related proteins, some of the FH family proteins are devoted to down-regulating the AP, while others exert an opposite function by promoting AP activation. Recent findings have provided insights into the molecular mechanisms defining their biological roles and their pathogenicity, illustrating the relevance that the balance between the regulators and the activators within this protein family has in defining the outcome of complement activation on cell surfaces. In this review we will discuss the emerging roles of the factor H protein family, their impact in the complement cascade, and their involvement in the pathogenesis of complement-mediated diseases associated with the AP dysregulation.

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Dates
Created: 2022Issued: 2024-01-16Updated: 2024-01-08
Relations
Is part of: es/2024/0401
DOI
https://doi.org/10.1111/imr.13166
Faculty
Medizin
Publisher
Philipps-Universität Marburg
Language
eng
Data types
Article
Keywords
immunologyimmunologyalternative pathwayalternative pathwayFactor Hcomplement systemcomplement systemFactor H
DDC-Numbers
570
License
https://creativecommons.org/licenses/by-nc-nd/4.0/
URI
https://open.uni-marburg.de/handle/10.17192/es.2024.0402
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Lucientes-Continente, Laura (0000-0001-5596-370X); Márquez-Tirado, Bárbara (0000-0001-7017-5031); Goicoechea de Jorge, Elena (0000-0002-4978-2483): The Factor H protein family: The switchers of the complement alternative pathway. In: , Jg. (2024-01-16), . DOI: https://doi.org/10.1111/imr.13166.

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Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 - CC BY NC ND
Except where otherwised noted, this item's license is described as Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 - CC BY NC ND