ArticleJournal of biomolecular screening2013
A high-throughput cell-based Gaussia luciferase reporter assay for identifying modulators of fibulin-3 secretion.
Article in Journal of biomolecular screening, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 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.
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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
21 citing papers in PubMed, 32 citations in OpenAlex.
- Genetic removal of Nlrp3 protects against age-related and R345W Efemp1-induced basal laminar deposit formation.Cell death & disease · 2025Article
- A spectrum of cysteine mutations in fibulin-3 highlight its disulfide bonding complexity and potential to induce ER stress.Scientific reports · 2025Article
- Protocol for HiBiT tagging endogenous proteins using CRISPR-Cas9 gene editing.STAR protocols · 2024Article
- Chemical interference with DSIF complex formation lowers synthesis of mutant huntingtin gene products and curtails mutant phenotypes.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- A Simple Secretion Assay for Assessing New and Existing Myocilin Variants.Current eye research · 2022Article
- Clinically-identified C-terminal mutations in fibulin-3 are prone to misfolding and destabilization.Scientific reports · 2021Article
- A novel homozygous missense mutation p.P388S inMolecular vision · 2021Article
- Fibulin-3 knockout mice demonstrate corneal dysfunction but maintain normal retinal integrity.Journal of molecular medicine (Berlin, Germany) · 2020Article
- Expression of R345W-Fibulin-3 Induces Epithelial-Mesenchymal Transition in Retinal Pigment Epithelial Cells.Frontiers in cell and developmental biology · 2020Article
- Non-antibiotic Small-Molecule Regulation of DHFR-Based Destabilizing DomainsMolecular therapy. Methods & clinical development · 2019Article
- Measuring Relative Insulin Secretion using a Co-Secreted Luciferase Surrogate.Journal of visualized experiments : JoVE · 2019Article
- Fibulin-3 serum and urine levels in the diagnosis and severity assessment of primary knee osteoarthritis.Reumatologia · 2019Article
- A High-Throughput Assay for Collagen Secretion Suggests an Unanticipated Role for Hsp90 in Collagen Production.Biochemistry · 2018Article
- Liquid Biopsy of Vitreous Reveals an Abundant Vesicle Population Consistent With the Size and Morphology of Exosomes.Translational vision science & technology · 2018Article
- Conditional, Genetically Encoded, Small Molecule-Regulated Inhibition of NFκB Signaling in RPE Cells.Investigative ophthalmology & visual science · 2017Article
- A Novel Luciferase Assay For Sensitively Monitoring Myocilin Variants in Cell Culture.Investigative ophthalmology & visual science · 2016Article
- Evidence of Alternative Cystatin C Signal Sequence Cleavage Which Is Influenced by the A25T Polymorphism.PloS one · 2016Article
- Targeting HER3 by interfering with its Sec61-mediated cotranslational insertion into the endoplasmic reticulum.Oncogene · 2015Article
- Genetic ablation of N-linked glycosylation reveals two key folding pathways for R345W fibulin-3, a secreted protein associated with retinal degeneration.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2015Article
- Differential tolerance of 'pseudo-pathogenic' tryptophan residues in calcium-binding EGF domains of short fibulin proteins.Experimental eye research · 2015Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
An R345W mutation in fibulin-3 causes its inefficient secretion, increased intracellular steady-state levels, and the macular dystrophy, Malattia Leventinese (ML), a disease similar to age-related macular degeneration. It is unknown whether R345W causes ML through increased intracellular levels, by the secretion of a potentially aggregation-prone protein, or both. To identify small molecules that alter the secretion of fibulin-3, we developed ARPE19 retinal cell lines that inducibly express wild-type (WT) or R345W fibulin-3 fused to an enhanced Gaussia luciferase (eGLuc2). Screening of the Library of Pharmacologically Active Compounds demonstrated that these cell lines and the GLuc assay are suitable for high-throughput chemical screening. Two estrogen-related compounds enhanced fibulin-3 secretion, whereas a diverse series of small molecules reduced fibulin-3 secretion. A counterscreen identified compounds that did not substantially alter the secretion of unfused eGLuc2, demonstrating at least partial selectivity for fibulin-3. A secondary assay using untagged fibulin-3 confirmed that the top three inhibitory compounds reduced R345W fibulin-3 secretion. Interestingly, in untagged fibulin-3 studies, one compound, phorbol 12-myristate 13-acetate, reduced R345W fibulin-3 secretion while minimally enhancing WT fibulin-3 secretion, the desired activity and selectivity we sought for ML. The identified compounds could serve as tools for probing the etiology of fibulin-3-related diseases.
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Registered trials
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