ArticleBlood2014
A Caenorhabditis elegans-based assay recognizes immunoglobulin light chains causing heart amyloidosis.
Article in Blood, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04392960 (Molecular, Magnetic Resonance, and Echocardiographic Imaging Combined With Biomarkers of Cardiac and Clonal Disease to Predict Survival and Assess Response to Therapy in Cardiac AL Amyloidosis), which is not on this map. Cited by 66 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.
Molecular, Magnetic Resonance, and Echocardiographic Imaging Combined With Biomarkers of Cardiac and Clonal Disease to Predict Survival and Assess Response to Therapy in Cardiac AL Amyloidosis
Who cites it
66 citing papers in PubMed, 158 citations in OpenAlex.
- Phase 2 trial of ixazomib, cyclophosphamide, and dexamethasone for previously untreated light chain amyloidosis.Blood advances · 2022Trial
- Phase 2 trial of daily, oral epigallocatechin gallate in patients with light-chain amyloidosis.International journal of hematology · 2017Trial
- Imaging Advances in Light Chain Amyloidosis.Diagnostics (Basel, Switzerland) · 2026Review
- Utilization and Prognosis of Cardiac Device Implantation in AL Versus ATTR Amyloidosis.Pacing and clinical electrophysiology : PACE · 2026Article
- Mechanisms of damage and therapies for cardiac amyloidosis: a role for inflammation?Clinical research in cardiology : official journal of the German Cardiac Society · 2026Review
- A molecular perspective of gelsolin amyloidosis: An old foe with new faces.Cellular and molecular life sciences : CMLS · 2026Review
- Human-Induced Pluripotent Stem Cell Models for Amyloid Cardiomyopathy: From Mechanistic Insights to Therapeutic Discovery.Journal of cardiovascular development and disease · 2025Review
- Clonal plasma cell features in light chain amyloidosis are associated with depth and timing of cardiac response independent of hematologic response.Haematologica · 2025Article
- In vitro and in vivo disease models of cardiac amyloidosis: progress, pitfalls, and potential.Cardiovascular research · 2025Review
- Serum and Tissue Light-Chains as Disease Biomarkers in AL Amyloidosis.International journal of molecular sciences · 2025Review
- Modeling immunoglobulin light chain amyloidosis in Caenorhabditis elegans.Disease models & mechanisms · 2025Article
- A mouse model of cardiac immunoglobulin light chain amyloidosis reveals insights into tissue accumulation and toxicity of amyloid fibrils.Nature communications · 2025Article
- Amyloidogenic immunoglobulin light chains disturb contractile function and calcium transients in a human cardiac spheroid model of light chain (AL) amyloidosis.Scientific reports · 2025Article
- Case report: Light-chain amyloidosis responsive to selinexor in combination with daratumumab and dexamethasone (SDd) therapy.Frontiers in medicine · 2024Article
- Amyloidogenic light chains impair plasma cell survival.Haematologica · 2023Article
- Human wild-type and D76N βFASEB bioAdvances · 2023Article
- Predictors of treatment response and survival outcomes in patients with advanced cardiac AL amyloidosis.Blood advances · 2023Article
- Status and Future Directions of Therapeutics and Prognosis of Cardiac Amyloidosis.Therapeutics and clinical risk management · 2023Review
- Rational Design of a Peptidomimetic Inhibitor of Gelsolin Amyloid Aggregation.International journal of molecular sciences · 2022Article
- Noninvasive Diagnostics of Renal Amyloidosis: Current State and Perspectives.International journal of molecular sciences · 2022Review
6 more citing papers are in PubMed but not listed here.
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Authors and funding
15 authors at 5 institutions in 1 country.
Funding
Abstract
Poor prognosis and limited therapeutic options characterize immunoglobulin light-chain (AL) amyloidosis with major heart involvement. Reliable experimental models are needed to study light-chain (LC)/heart interactions and to explore strategies for prevention of cardiac damage. We have exploited the nematode Caenorhabditis elegans as a novel tool, because its pharynx is evolutionarily related to the vertebrate heart. Our data demonstrate that the pharyngeal pumping of C elegans is significantly and selectively reduced by LCs from AL patients suffering from cardiomyopathy, but not by amyloid LCs with different organ tropism or nonamyloidogenic LCs from multiple myeloma. This functional alteration is dependent on the LC concentration and results in persistent pharyngeal dysfunction and in a significant reduction of the worms' lifespan. These manifestations are paralleled by an increase of mitochondrial reactive oxygen species and can be prevented by treatment with antioxidant agents. In conclusion, these data indicate that this nematode-based assay is a promising surrogate model for investigating the heart-specific toxicity of amyloidogenic LCs and for a rapid screening of new therapeutic strategies.
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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.