The Experts below are selected from a list of 48 Experts worldwide ranked by ideXlab platform
Francesca Lavatelli - One of the best experts on this subject based on the ideXlab platform.
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novel mitochondrial protein interactors of immunoglobulin light chains causing Heart Amyloidosis
The FASEB Journal, 2015Co-Authors: Francesca Lavatelli, Esther Imperlini, Stefania Orru, Paola Rognoni, Daniela Sarnataro, G Palladini, Giuseppe Malpasso, Maria Eugenia Soriano, Andrea Di Fonzo, Veronica ValentiniAbstract:In immunoglobulin (Ig) light-chain (LC) (AL) Amyloidosis, AL deposition translates into life-threatening cardiomyopathy. Clinical and experimental evidence indicates that soluble cardiotoxic LCs ar...
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a caenorhabditis elegans based assay recognizes immunoglobulin light chains causing Heart Amyloidosis
Blood, 2014Co-Authors: Luisa Diomede, Francesca Lavatelli, Paola Rognoni, Andrea Di Fonzo, Margherita Romeo, Elena Del Favero, Laura Cantu, Elena Maria Ghibaudi, Alessandro Corbelli, Fabio FiordalisoAbstract: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.
Andrea Di Fonzo - One of the best experts on this subject based on the ideXlab platform.
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novel mitochondrial protein interactors of immunoglobulin light chains causing Heart Amyloidosis
The FASEB Journal, 2015Co-Authors: Francesca Lavatelli, Esther Imperlini, Stefania Orru, Paola Rognoni, Daniela Sarnataro, G Palladini, Giuseppe Malpasso, Maria Eugenia Soriano, Andrea Di Fonzo, Veronica ValentiniAbstract:In immunoglobulin (Ig) light-chain (LC) (AL) Amyloidosis, AL deposition translates into life-threatening cardiomyopathy. Clinical and experimental evidence indicates that soluble cardiotoxic LCs ar...
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a caenorhabditis elegans based assay recognizes immunoglobulin light chains causing Heart Amyloidosis
Blood, 2014Co-Authors: Luisa Diomede, Francesca Lavatelli, Paola Rognoni, Andrea Di Fonzo, Margherita Romeo, Elena Del Favero, Laura Cantu, Elena Maria Ghibaudi, Alessandro Corbelli, Fabio FiordalisoAbstract: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.
Paola Rognoni - One of the best experts on this subject based on the ideXlab platform.
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novel mitochondrial protein interactors of immunoglobulin light chains causing Heart Amyloidosis
The FASEB Journal, 2015Co-Authors: Francesca Lavatelli, Esther Imperlini, Stefania Orru, Paola Rognoni, Daniela Sarnataro, G Palladini, Giuseppe Malpasso, Maria Eugenia Soriano, Andrea Di Fonzo, Veronica ValentiniAbstract:In immunoglobulin (Ig) light-chain (LC) (AL) Amyloidosis, AL deposition translates into life-threatening cardiomyopathy. Clinical and experimental evidence indicates that soluble cardiotoxic LCs ar...
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a caenorhabditis elegans based assay recognizes immunoglobulin light chains causing Heart Amyloidosis
Blood, 2014Co-Authors: Luisa Diomede, Francesca Lavatelli, Paola Rognoni, Andrea Di Fonzo, Margherita Romeo, Elena Del Favero, Laura Cantu, Elena Maria Ghibaudi, Alessandro Corbelli, Fabio FiordalisoAbstract: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.
Veronica Valentini - One of the best experts on this subject based on the ideXlab platform.
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novel mitochondrial protein interactors of immunoglobulin light chains causing Heart Amyloidosis
The FASEB Journal, 2015Co-Authors: Francesca Lavatelli, Esther Imperlini, Stefania Orru, Paola Rognoni, Daniela Sarnataro, G Palladini, Giuseppe Malpasso, Maria Eugenia Soriano, Andrea Di Fonzo, Veronica ValentiniAbstract:In immunoglobulin (Ig) light-chain (LC) (AL) Amyloidosis, AL deposition translates into life-threatening cardiomyopathy. Clinical and experimental evidence indicates that soluble cardiotoxic LCs ar...
Fabio Fiordaliso - One of the best experts on this subject based on the ideXlab platform.
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a caenorhabditis elegans based assay recognizes immunoglobulin light chains causing Heart Amyloidosis
Blood, 2014Co-Authors: Luisa Diomede, Francesca Lavatelli, Paola Rognoni, Andrea Di Fonzo, Margherita Romeo, Elena Del Favero, Laura Cantu, Elena Maria Ghibaudi, Alessandro Corbelli, Fabio FiordalisoAbstract: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.
