The extracellular microenvironment in immune dysregulation and inflammation in retinal disorders

Frontiers in Immunology

Immunology

Karolina Plössl¹  Fabiola Biasella¹  Paul N. Baird²  Bernhard H. F. Weber³ 

[1] Institute of Human Genetics, University of Regensburg, Regensburg, Germany;Institute of Human Genetics, University of Regensburg, Regensburg, Germany;Department of Surgery, Ophthalmology, University of Melbourne, Melbourne, VIC, Australia;Institute of Human Genetics, University of Regensburg, Regensburg, Germany;Institute of Clinical Human Genetics, University Hospital Regensburg, Regensburg, Germany;

关键词: extracellular matrix;    Bruch’s membrane;    matricellular proteins;    inflammation;    retinal disease;    age-related macular degeneration;    Sorsby fundus dystrophy;    TIMP3;   

DOI  :  10.3389/fimmu.2023.1147037

 received in 2023-01-18, accepted in 2023-02-15,  发布年份 2023

来源: Frontiers

【 摘 要 】

Inherited retinal dystrophies (IRDs) as well as genetically complex retinal phenotypes represent a heterogenous group of ocular diseases, both on account of their phenotypic and genotypic characteristics. Therefore, overlaps in clinical features often complicate or even impede their correct clinical diagnosis. Deciphering the molecular basis of retinal diseases has not only aided in their disease classification but also helped in our understanding of how different molecular pathologies may share common pathomechanisms. In particular, these relate to dysregulation of two key processes that contribute to cellular integrity, namely extracellular matrix (ECM) homeostasis and inflammation. Pathological changes in the ECM of Bruch’s membrane have been described in both monogenic IRDs, such as Sorsby fundus dystrophy (SFD) and Doyne honeycomb retinal dystrophy (DHRD), as well as in the genetically complex age-related macular degeneration (AMD) or diabetic retinopathy (DR). Additionally, complement system dysfunction and distorted immune regulation may also represent a common connection between some IRDs and complex retinal degenerations. Through highlighting such overlaps in molecular pathology, this review aims to illuminate how inflammatory processes and ECM homeostasis are linked in the healthy retina and how their interplay may be disturbed in aging as well as in disease.

【 授权许可】

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Copyright © 2023 Biasella, Plössl, Baird and Weber