Can Novel Treatment of Age-Related MacularDegeneration Be Developed by Better Understanding of Sorsby’s Fundus Dystrophy

Journal of Clinical Medicine

N. Victor Chong¹  Hanae C. Y. Gourier¹ 

[1] Oxford Eye Hospital, Oxford University Hospitals, Oxford, OX3 9DU, UK;

关键词: Sorsby’s fundus dystrophy;    age-related macular degeneration;    Bruch’s membrane;    TIMP-3;    choroidal neovascularisation;    geographic atrophy;   

DOI  :  10.3390/jcm4050874

来源: DOAJ

【 摘 要 】

Sorsby’s Fundus Dystrophy (SFD) is a rare autosomal dominant maculopathy that shares many clinical features with Age-Related Macular Degeneration (AMD). It is caused by a mutation in a single gene, TIMP-3, which accumulates in Bruch’s membrane (BM). BM thickening and TIMP-3 accumulation can also be found in AMD. From our understanding of the pathophysiology of SFD we hypothesize that BM thickening could be responsible for making the elastic layer vulnerable to invasion by choriocapillaris, thereby leading to choroidal neovascularization in some cases of AMD, whilst in others it could deprive the retinal pigment epithelium of its blood supply, thereby causing geographic atrophy.

【 授权许可】

Unknown