ArticleThe American journal of pathology2013
Intracellularly-retained decorin lacking the C-terminal ear repeat causes ER stress: a cell-based etiological mechanism for congenital stromal corneal dystrophy.
Article in The American journal of pathology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed, 30 citations in OpenAlex.
- The Role of Decorin in the Ocular Surface: Structure, Function, and Therapeutic Potential.Proteoglycan research · 2025Article
- Decorin: matrix-based pan-cancer tumor suppressor.Molecular and cellular biochemistry · 2025Review
- Global impact of proteoglycan science on human diseases.iScience · 2023Review
- Endoplasmic reticulum stress: molecular mechanism and therapeutic targets.Signal transduction and targeted therapy · 2023Review
- A pediatric case of congenital stromal corneal dystrophy caused by the novel variant c.953del of the DCN gene.Human genome variation · 2023Article
- Oncosuppressive roles of decorin through regulation of multiple receptors and diverse signaling pathways.American journal of physiology. Cell physiology · 2022Review
- Sequence features, structure, ligand interaction, and diseases in small leucine rich repeat proteoglycans.Journal of cell communication and signaling · 2021Review
- Six-Month In Vivo Safety Profiling of Topical Ocular AAV5-Decorin Gene Transfer.Translational vision science & technology · 2021Article
- Composition, structure and function of the corneal stroma.Experimental eye research · 2020Review
- From Translation to Protein Degradation as Mechanisms for Regulating Biological Functions: A Review on the SLRP Family in Skeletal Tissues.Biomolecules · 2020Review
- Novel Mutations inFrontiers in cell and developmental biology · 2020Article
- Generation of a multi-functional, target organ-specific, anti-fibrotic molecule by molecular engineering of the extracellular matrix protein, decorin.British journal of pharmacology · 2019Review
- Small leucine-rich repeat proteoglycans in corneal inflammation and wound healing.Experimental eye research · 2016Review
- Decorin as a multivalent therapeutic agent against cancer.Advanced drug delivery reviews · 2016Review
- Characteristics of corneal dystrophies: a review from clinical, histological and genetic perspectives.International journal of ophthalmology · 2016Review
- Regulation of corneal stroma extracellular matrix assembly.Experimental eye research · 2015Review
- Proteoglycan form and function: A comprehensive nomenclature of proteoglycans.Matrix biology : journal of the International Society for Matrix Biology · 2015Review
- Article
- Decorin: A Growth Factor Antagonist for Tumor Growth Inhibition.BioMed research international · 2015Review
- Decorin differentially modulates the activity of insulin receptor isoform A ligands.Matrix biology : journal of the International Society for Matrix Biology · 2014Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
Abstract
Decorin, a small leucine-rich proteoglycan (SLRP), is involved in the pathophysiology of human congenital stromal corneal dystrophy (CSCD). This disease is characterized by corneal opacities and vision impairment. In reported cases, the human gene encoding decorin contains point mutations in exon 10, generating a truncated form of decorin lacking the C-terminal 33 amino acid residues. We have previously described a transgenic mouse model carrying a similar mutation in the decorin gene that leads to an ocular phenotype characterized by corneal opacities identical to CSCD in humans. We have also identified abnormal synthesis and secretion of various SLRPs in mutant mouse corneas. In the present study, we found that mutant C-terminal truncated decorin was retained in the cytoplasm of mouse keratocytes in vivo and of transfected human embryonic kidney cells. This resulted in endoplasmic reticulum stress and an unfolded protein response. Thus, we propose a novel cell-based mechanism underlying CSCD in which a truncated SLRP protein core is retained intracellularly, its accumulation triggering endoplasmic reticulum stress that results in abnormal SLRP synthesis and secretion, which ultimately affects stromal structure and corneal transparency.
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What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.