Academic Journal
Προβολή σε EDS

Decoding the Factor H-Related Proteins: Gatekeepers of Complement Dysregulation in AMD.

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος: Decoding the Factor H-Related Proteins: Gatekeepers of Complement Dysregulation in AMD.
Συγγραφείς: Tang J; Institute for Ophthalmic Research, Department for Ophthalmology, Eberhard Karls University of Tübingen, Tübingen, Germany., Zgoda N; Institute for Ophthalmic Research, Department for Ophthalmology, Eberhard Karls University of Tübingen, Tübingen, Germany., Clark SJ; Institute for Ophthalmic Research, Department for Ophthalmology, Eberhard Karls University of Tübingen, Tübingen, Germany.; University Eye Clinic, Eberhard Karls University of Tübingen, Tübingen, Germany.; Lydia Becker Institute of Immunology and Inflammation, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
Πηγή: European journal of immunology [Eur J Immunol] 2026 Jun; Vol. 56 (6), pp. e70228.
Τύπος έκδοσης: Journal Article; Review
Γλώσσα: English
Στοιχεία περιοδικού: Publisher: Wiley-VCH Country of Publication: Germany NLM ID: 1273201 Publication Model: Print Cited Medium: Internet ISSN: 1521-4141 (Electronic) Linking ISSN: 00142980 NLM ISO Abbreviation: Eur J Immunol Subsets: MEDLINE
Imprint Name(s): Publication: <2005->: Weinheim : Wiley-VCH
Original Publication: Weinheim, Verlag Chemie GmbH.
Ιατρικοί όροι (MeSH): Macular Degeneration*/immunology , Macular Degeneration*/genetics , Complement System Proteins*/immunology , Complement Factor H*/immunology , Complement Factor H*/genetics , Blood Proteins*/genetics , Blood Proteins*/immunology , Blood Proteins*/metabolism, Complement Activation/immunology ; Inflammation/immunology ; Complement C3b Inactivator Proteins/genetics ; Humans ; Animals
Περίληψη: Age-related macular degeneration (AMD) is the third most common form of blindness in the Western world, with a predicted 288 million individuals affected worldwide by the year 2040. Both genetic and biochemical evidence point toward complement overactivation, through a diminished regulatory capacity, at the back of the eye, causing inflammation and tissue damage that helps drive this devastating disease. While much historic effort has gone into understanding the loss of regulatory control by complement factor H and factor H-like protein 1, recent studies have uncovered an emerging role of the factor H-related proteins and their capacity for driving forward complement amplification. FHR gene deletions have been shown to be protective against AMD, and increased circulating levels of FHR proteins have been found to associate with their deposition in the back of the eye at the site of disease pathogenesis. Here, we will explore the current understanding of FHR and their association with AMD risk, possible mechanisms by which they promote inflammation and extracellular matrix remodeling, and the potential effectiveness of their targeting as a novel therapeutic strategy for reducing the risk of AMD.
(© 2026 The Author(s). European Journal of Immunology published by Wiley‐VCH GmbH.)
References: Cell Mol Life Sci. 2021 May;78(10):4487-4505. (PMID: 33751148)
Eur J Immunol. 1991 Mar;21(3):799-802. (PMID: 1826264)
Br J Cancer. 2002 Nov 4;87(10):1119-27. (PMID: 12402151)
J Immunol. 2005 May 15;174(10):6250-6. (PMID: 15879123)
JAMA. 2024 Jan 9;331(2):147-157. (PMID: 38193957)
Nat Commun. 2024 Jan 10;15(1):443. (PMID: 38200010)
Invest Ophthalmol Vis Sci. 2015 Jul;56(8):4870-9. (PMID: 26218915)
Invest Ophthalmol Vis Sci. 2011 Aug 17;52(9):6511-21. (PMID: 21746802)
Front Immunol. 2017 Sep 19;8:1145. (PMID: 28974948)
Nat Genet. 2010 Jul;42(7):553-5; author reply 555-6. (PMID: 20581873)
Nat Commun. 2021 Dec 15;12(1):7293. (PMID: 34911940)
Nat Genet. 2006 Oct;38(10):1173-7. (PMID: 16998489)
Eur J Immunol. 1987 Oct;17(10):1485-9. (PMID: 2445583)
Pflugers Arch. 2007 Jun;454(3):345-59. (PMID: 17256154)
J Biol Chem. 2010 Sep 24;285(39):30192-202. (PMID: 20660596)
Nat Genet. 2016 Feb;48(2):134-43. (PMID: 26691988)
Dis Model Mech. 2015 May;8(5):421-7. (PMID: 26035859)
Front Immunol. 2017 Dec 19;8:1778. (PMID: 29312308)
Front Immunol. 2013 Nov 26;4:412. (PMID: 24324472)
J Neuroinflammation. 2025 Jul 3;22(1):173. (PMID: 40611130)
Nat Genet. 2011 Oct 23;43(12):1232-6. (PMID: 22019782)
J Immunol. 2015 May 15;194(10):4963-73. (PMID: 25855355)
Sci Rep. 2018 Feb 14;8(1):3040. (PMID: 29445097)
Ophthalmology. 2018 Jul;125(7):1064-1074. (PMID: 29398083)
Front Immunol. 2021 Mar 30;12:660194. (PMID: 33868311)
Hum Mol Genet. 2010 Jun 15;19(12):2468-86. (PMID: 20360305)
Nat Rev Nephrol. 2016 Jul;12(7):383-401. (PMID: 27211870)
Methods Mol Biol. 2021;2227:159-178. (PMID: 33847941)
Cell Mol Life Sci. 2017 May;74(9):1605-1624. (PMID: 27942748)
Front Immunol. 2015 Jun 02;6:262. (PMID: 26082779)
Hum Mol Genet. 2014 Aug 1;23(15):4001-14. (PMID: 24634144)
Front Immunol. 2016 Nov 28;7:542. (PMID: 27965669)
J Innate Immun. 2014;6(4):407-16. (PMID: 24335201)
Pharmacol Rev. 2021 Apr;73(2):792-827. (PMID: 33687995)
JCI Insight. 2020 Jan 16;5(1):. (PMID: 31829999)
Front Immunol. 2021 Nov 08;12:769242. (PMID: 34819935)
Nat Commun. 2025 Jul 1;16(1):5766. (PMID: 40593839)
Eur J Hum Genet. 2005 Mar;13(3):321-9. (PMID: 15562282)
Front Immunol. 2021 Aug 18;12:676662. (PMID: 34489931)
N Engl J Med. 2001 Apr 5;344(14):1058-66. (PMID: 11287977)
Biochem J. 1989 Mar 15;258(3):927-30. (PMID: 2525027)
Semin Immunopathol. 2021 Dec;43(6):757-771. (PMID: 34698894)
Hum Mol Genet. 2008 Apr 1;17(7):971-7. (PMID: 18084039)
Biochem J. 1988 Jan 15;249(2):593-602. (PMID: 2963625)
Glycobiology. 2007 Sep;17(9):932-44. (PMID: 17591618)
Nat Commun. 2020 Feb 7;11(1):778. (PMID: 32034129)
Am J Hum Genet. 2021 Aug 5;108(8):1367-1384. (PMID: 34260947)
Front Immunol. 2018 Jul 12;9:1607. (PMID: 30050540)
FEBS Lett. 1999 Dec 3;462(3):345-52. (PMID: 10622723)
Mol Vis. 2013 Nov 16;19:2274-97. (PMID: 24265543)
Int Rev Immunol. 1993;10(1):65-86. (PMID: 8340678)
Hum Mol Genet. 2010 Dec 1;19(23):4694-704. (PMID: 20843825)
Exp Eye Res. 2013 Jun;111:105-11. (PMID: 23500522)
Mol Oncol. 2019 Dec;13(12):2531-2553. (PMID: 31365168)
Lancet Glob Health. 2014 Feb;2(2):e106-16. (PMID: 25104651)
J Exp Med. 1985 May 1;161(5):1189-95. (PMID: 3157763)
Mol Immunol. 1999 Sep-Oct;36(13-14):803-8. (PMID: 10698333)
FASEB J. 2006 Jan;20(1):9-22. (PMID: 16394262)
J Biol Chem. 2012 Jun 1;287(23):19528-36. (PMID: 22518841)
Front Immunol. 2017 Oct 18;8:1328. (PMID: 29093712)
Science. 2005 Apr 15;308(5720):421-4. (PMID: 15761121)
Am J Hum Genet. 2001 Feb;68(2):478-84. (PMID: 11170895)
Nat Chem Biol. 2015 Jan;11(1):77-82. (PMID: 25402769)
Front Immunol. 2015 Feb 02;6:25. (PMID: 25699044)
Immunogenetics. 1988;27(3):211-4. (PMID: 2962936)
J Immunol. 2016 Nov 1;197(9):3471-3480. (PMID: 27671112)
PLoS One. 2013 Nov 18;8(11):e78617. (PMID: 24260121)
Biosci Rep. 1987 Mar;7(3):201-7. (PMID: 2889480)
Semin Immunopathol. 2018 Jan;40(1):65-74. (PMID: 28948331)
Proc Natl Acad Sci U S A. 2013 Mar 19;110(12):4685-90. (PMID: 23487775)
J Immunol. 2017 Jul 1;199(1):292-303. (PMID: 28533443)
Mol Immunol. 2009 Jan;46(3):335-44. (PMID: 19084272)
Am J Hum Genet. 2021 Aug 5;108(8):1385-1400. (PMID: 34260948)
Science. 2015 May 8;348(6235):648-60. (PMID: 25954001)
Genomics. 2022 Mar;114(2):110286. (PMID: 35124170)
Front Immunol. 2018 Apr 17;9:729. (PMID: 29719534)
J Biol Chem. 1997 Feb 28;272(9):5627-34. (PMID: 9038172)
J Immunol. 2000 Jun 1;164(11):6075-81. (PMID: 10820293)
Sci Rep. 2026 Mar 22;16(1):. (PMID: 41866380)
Grant Information: China Scholarship Council; Helmut Ecker Foundation; 538134976 DFG
Substance Nomenclature: 9007-36-7 (Complement System Proteins)
80295-65-4 (Complement Factor H)
0 (Blood Proteins)
0 (factor H-related protein 1)
0 (Complement C3b Inactivator Proteins)
Entry Date(s): Date Created: 20260617 Date Completed: 20260617 Latest Revision: 20260813
Update Code: 20260813
PubMed Central ID: PMC13273927
DOI: 10.1002/eji.70228
PMID: 42307030
Βάση Δεδομένων: MEDLINE
Περιγραφή
ISSN:1521-4141
DOI:10.1002/eji.70228