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Article

Accumulation of tissue inhibitor of metalloproteinases-3 in human eyes with Sorsby's fundus dystrophy or retinitis pigmentosa.

The British journal of ophthalmology - 1 Nov 1998

Fariss R N, Apte S S, Luthert P J, Bird A C, Milam A H

Abstract excerpt

BACKGROUND/AIMS: Tissue inhibitor of metalloproteinases-3 (TIMP-3) is normally synthesised by the retinal pigment epithelium (RPE) and deposited in Bruch's membrane. Mutations in the TIMP3 gene cause Sorsby's fundus dystrophy (SFD), which is characterised by thickening of Bruch's membrane, choroidal neovascularisation, and photoreceptor degeneration. To elucidate the role of TIMP-3 in human retinal degenerative...

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Source

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Identifiers

PMID: 9924344
DOI: 10.1136/bjo.82.11.1329
PMCID: PMC1722431

Genes

RPE
TIMP3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Accumulation of tissue inhibitor of metalloproteinases-3 in human eyes with Sorsby's fundus dystrophy or retinitis pigmentosa.PMID 9924344Read ↗

Sorsby fundus dystrophy: Insights from the past and looking to the future.Semantic proximity Read ↗

TIMP-3, collagen, and elastin immunohistochemistry and histopathology of Sorsby's fundus dystrophy.Semantic proximity Read ↗

A novel tissue inhibitor of metalloproteinases-3 mutation reveals a common molecular phenotype in Sorsby's fundus dystrophy.Semantic proximity Read ↗

Review: Mechanisms of TIMP-3 accumulation and pathogenesis in Sorsby fundus dystrophy.Semantic proximity Read ↗

Sorsby's fundus dystrophy mutations impair turnover of TIMP-3 by retinal pigment epithelial cells.Semantic proximity Read ↗

Sorsby fundus dystrophy mutation Timp3(S156C) affects the morphological and biochemical phenotype but not metalloproteinase homeostasis.Semantic proximity Read ↗

The Diverse Roles of TIMP-3: Insights into Degenerative Diseases of the Senescent Retina and Brain.Semantic proximity Read ↗

TIMP3 mutation in Sorsby's fundus dystrophy: molecular insights.Semantic proximity Read ↗

Sorsby Fundus Dystrophy Mutation in Tissue Inhibitor of Metalloproteinase 3 (TIMP3) promotes Choroidal Neovascularization via a Fibroblast Growth Factor-dependent Mechanism.Semantic proximity Read ↗

A mouse model for Sorsby fundus dystrophySemantic proximity Read ↗

In vitro stem cell modelling demonstrates a proof-of-concept for excess functional mutant TIMP3 as the cause of Sorsby fundus dystrophy.Semantic proximity Read ↗

Genetic aberrations in tissue inhibitor of metalloproteinases-3 lead to manifestations of the myofibroblast phenotype in mouse fibroblasts and fibroblasts of patients with Sorsby fundus dystrophy.Semantic proximity Read ↗

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