Heart failure after pressure overload in autosomal-dominant desminopathies: Lessons from heterozygous DES-p.R349P knock-in mice
Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the...
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| Published in: | PloS one Vol. 15; no. 3; p. e0228913 |
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| Abstract | Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the frequent human DES missense mutation p.R350P.
Transverse aortic constriction (TAC) was performed on heterozygous (HET) DES-p.R349P mice and wild-type (WT) littermates. Echocardiography demonstrated reduced left ventricular ejection fraction in HET-TAC (WT-sham: 69.5 ± 2.9%, HET-sham: 64.5 ± 4.7%, WT-TAC: 63.5 ± 4.9%, HET-TAC: 55.7 ± 5.4%; p<0.01). Cardiac output was significantly reduced in HET-TAC (WT sham: 13088 ± 2385 μl/min, HET sham: 10391 ± 1349μl/min, WT-TAC: 8097 ± 1903μl/min, HET-TAC: 5793 ± 2517μl/min; p<0.01). Incidence and duration of AV blocks as well as the probability to induce ventricular tachycardias was highest in HET-TAC. We observed reduced mtDNA copy numbers in HET-TAC (WT-sham: 12546 ± 406, HET-sham: 13526 ± 781, WT-TAC: 11155 ± 3315, HET-TAC: 8649 ± 1582; p = 0.025), but no mtDNA deletions. The activity of respiratory chain complexes I and IV showed the greatest reductions in HET-TAC.
Pressure overload in HET mice aggravated the clinical phenotype of cardiomyopathy and resulted in mitochondrial dysfunction. Preventive avoidance of pressure overload/arterial hypertension in desminopathy patients might represent a crucial therapeutic measure. |
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| AbstractList | BACKGROUNDMutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the frequent human DES missense mutation p.R350P. METHODS AND RESULTSTransverse aortic constriction (TAC) was performed on heterozygous (HET) DES-p.R349P mice and wild-type (WT) littermates. Echocardiography demonstrated reduced left ventricular ejection fraction in HET-TAC (WT-sham: 69.5 ± 2.9%, HET-sham: 64.5 ± 4.7%, WT-TAC: 63.5 ± 4.9%, HET-TAC: 55.7 ± 5.4%; p<0.01). Cardiac output was significantly reduced in HET-TAC (WT sham: 13088 ± 2385 μl/min, HET sham: 10391 ± 1349μl/min, WT-TAC: 8097 ± 1903μl/min, HET-TAC: 5793 ± 2517μl/min; p<0.01). Incidence and duration of AV blocks as well as the probability to induce ventricular tachycardias was highest in HET-TAC. We observed reduced mtDNA copy numbers in HET-TAC (WT-sham: 12546 ± 406, HET-sham: 13526 ± 781, WT-TAC: 11155 ± 3315, HET-TAC: 8649 ± 1582; p = 0.025), but no mtDNA deletions. The activity of respiratory chain complexes I and IV showed the greatest reductions in HET-TAC. CONCLUSIONPressure overload in HET mice aggravated the clinical phenotype of cardiomyopathy and resulted in mitochondrial dysfunction. Preventive avoidance of pressure overload/arterial hypertension in desminopathy patients might represent a crucial therapeutic measure. Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the frequent human DES missense mutation p.R350P. Transverse aortic constriction (TAC) was performed on heterozygous (HET) DES-p.R349P mice and wild-type (WT) littermates. Echocardiography demonstrated reduced left ventricular ejection fraction in HET-TAC (WT-sham: 69.5 ± 2.9%, HET-sham: 64.5 ± 4.7%, WT-TAC: 63.5 ± 4.9%, HET-TAC: 55.7 ± 5.4%; p<0.01). Cardiac output was significantly reduced in HET-TAC (WT sham: 13088 ± 2385 [mu]l/min, HET sham: 10391 ± 1349[mu]l/min, WT-TAC: 8097 ± 1903[mu]l/min, HET-TAC: 5793 ± 2517[mu]l/min; p<0.01). Incidence and duration of AV blocks as well as the probability to induce ventricular tachycardias was highest in HET-TAC. We observed reduced mtDNA copy numbers in HET-TAC (WT-sham: 12546 ± 406, HET-sham: 13526 ± 781, WT-TAC: 11155 ± 3315, HET-TAC: 8649 ± 1582; p = 0.025), but no mtDNA deletions. The activity of respiratory chain complexes I and IV showed the greatest reductions in HET-TAC. Pressure overload in HET mice aggravated the clinical phenotype of cardiomyopathy and resulted in mitochondrial dysfunction. Preventive avoidance of pressure overload/arterial hypertension in desminopathy patients might represent a crucial therapeutic measure. Background Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the frequent human DES missense mutation p.R350P. Methods and results Transverse aortic constriction (TAC) was performed on heterozygous (HET) DES-p.R349P mice and wild-type (WT) littermates. Echocardiography demonstrated reduced left ventricular ejection fraction in HET-TAC (WT-sham: 69.5 ± 2.9%, HET-sham: 64.5 ± 4.7%, WT-TAC: 63.5 ± 4.9%, HET-TAC: 55.7 ± 5.4%; p<0.01). Cardiac output was significantly reduced in HET-TAC (WT sham: 13088 ± 2385 [mu]l/min, HET sham: 10391 ± 1349[mu]l/min, WT-TAC: 8097 ± 1903[mu]l/min, HET-TAC: 5793 ± 2517[mu]l/min; p<0.01). Incidence and duration of AV blocks as well as the probability to induce ventricular tachycardias was highest in HET-TAC. We observed reduced mtDNA copy numbers in HET-TAC (WT-sham: 12546 ± 406, HET-sham: 13526 ± 781, WT-TAC: 11155 ± 3315, HET-TAC: 8649 ± 1582; p = 0.025), but no mtDNA deletions. The activity of respiratory chain complexes I and IV showed the greatest reductions in HET-TAC. Conclusion Pressure overload in HET mice aggravated the clinical phenotype of cardiomyopathy and resulted in mitochondrial dysfunction. Preventive avoidance of pressure overload/arterial hypertension in desminopathy patients might represent a crucial therapeutic measure. BACKGROUND:Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the frequent human DES missense mutation p.R350P. METHODS AND RESULTS:Transverse aortic constriction (TAC) was performed on heterozygous (HET) DES-p.R349P mice and wild-type (WT) littermates. Echocardiography demonstrated reduced left ventricular ejection fraction in HET-TAC (WT-sham: 69.5 ± 2.9%, HET-sham: 64.5 ± 4.7%, WT-TAC: 63.5 ± 4.9%, HET-TAC: 55.7 ± 5.4%; p<0.01). Cardiac output was significantly reduced in HET-TAC (WT sham: 13088 ± 2385 μl/min, HET sham: 10391 ± 1349μl/min, WT-TAC: 8097 ± 1903μl/min, HET-TAC: 5793 ± 2517μl/min; p<0.01). Incidence and duration of AV blocks as well as the probability to induce ventricular tachycardias was highest in HET-TAC. We observed reduced mtDNA copy numbers in HET-TAC (WT-sham: 12546 ± 406, HET-sham: 13526 ± 781, WT-TAC: 11155 ± 3315, HET-TAC: 8649 ± 1582; p = 0.025), but no mtDNA deletions. The activity of respiratory chain complexes I and IV showed the greatest reductions in HET-TAC. CONCLUSION:Pressure overload in HET mice aggravated the clinical phenotype of cardiomyopathy and resulted in mitochondrial dysfunction. Preventive avoidance of pressure overload/arterial hypertension in desminopathy patients might represent a crucial therapeutic measure. Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We analyzed the effects of vascular pressure overload in a patient-mimicking p.R349P desmin knock-in mouse model that harbors the orthologue of the frequent human DES missense mutation p.R350P. Transverse aortic constriction (TAC) was performed on heterozygous (HET) DES-p.R349P mice and wild-type (WT) littermates. Echocardiography demonstrated reduced left ventricular ejection fraction in HET-TAC (WT-sham: 69.5 ± 2.9%, HET-sham: 64.5 ± 4.7%, WT-TAC: 63.5 ± 4.9%, HET-TAC: 55.7 ± 5.4%; p<0.01). Cardiac output was significantly reduced in HET-TAC (WT sham: 13088 ± 2385 μl/min, HET sham: 10391 ± 1349μl/min, WT-TAC: 8097 ± 1903μl/min, HET-TAC: 5793 ± 2517μl/min; p<0.01). Incidence and duration of AV blocks as well as the probability to induce ventricular tachycardias was highest in HET-TAC. We observed reduced mtDNA copy numbers in HET-TAC (WT-sham: 12546 ± 406, HET-sham: 13526 ± 781, WT-TAC: 11155 ± 3315, HET-TAC: 8649 ± 1582; p = 0.025), but no mtDNA deletions. The activity of respiratory chain complexes I and IV showed the greatest reductions in HET-TAC. Pressure overload in HET mice aggravated the clinical phenotype of cardiomyopathy and resulted in mitochondrial dysfunction. Preventive avoidance of pressure overload/arterial hypertension in desminopathy patients might represent a crucial therapeutic measure. |
| Audience | Academic |
| Author | Radecke, Tobias Kamm, Lisa Clemen, Christoph S Schröder, Rolf Eichhorn, Lars Kunz, Wolfram S Knappe, Vincent Peeva, Viktoriya Stöckigt, Florian Schultheis, Dorothea Kudin, Alexei P Beiert, Thomas Schrickel, Jan W Steinmetz, Martin Schlötzer-Schrehardt, Ursula Nickenig, Georg Berwanger, Carolin |
| AuthorAffiliation | Heart and Diabetes Center NRW, UNiversity Hospital of the Ruhr-University Bochum, GERMANY 6 Department of Epileptology, University Hospital of Bonn, Bonn, Germany 4 Department of Cardiology, University Hospital Essen, Hufelandstraße, Essen, Germany 5 Institute of Experimental Epileptology and Cognition Research, Bonn, Germany 3 Department of Anesthesiology, University Hospital Bonn, Bonn, Germany 9 Department of Opthalmology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany 7 Institute of Aerospace Medicine, German Aerospace Center (DLR), Linder Höhe, Cologne, Germany 2 Department of Cardiology, Krankenhaus Porz, Urbacher Weg, Cologne, Germany 8 Institute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany 10 Center for Biochemistry, Institute of Biochemistry I, Medical Faculty, University of Cologne, Cologne, Germany 11 Center for Physiology and Pat |
| AuthorAffiliation_xml | – name: 6 Department of Epileptology, University Hospital of Bonn, Bonn, Germany – name: 8 Institute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany – name: 1 Department of Cardiology, University Hospital Bonn, Bonn, Germany – name: 10 Center for Biochemistry, Institute of Biochemistry I, Medical Faculty, University of Cologne, Cologne, Germany – name: 11 Center for Physiology and Pathophysiology, Institute of Vegetative Physiology, Medical Faculty, University of Cologne, Cologne, Germany – name: Heart and Diabetes Center NRW, UNiversity Hospital of the Ruhr-University Bochum, GERMANY – name: 5 Institute of Experimental Epileptology and Cognition Research, Bonn, Germany – name: 2 Department of Cardiology, Krankenhaus Porz, Urbacher Weg, Cologne, Germany – name: 3 Department of Anesthesiology, University Hospital Bonn, Bonn, Germany – name: 4 Department of Cardiology, University Hospital Essen, Hufelandstraße, Essen, Germany – name: 9 Department of Opthalmology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany – name: 7 Institute of Aerospace Medicine, German Aerospace Center (DLR), Linder Höhe, Cologne, Germany |
| Author_xml | – sequence: 1 givenname: Florian orcidid: 0000-0002-0885-679X surname: Stöckigt fullname: Stöckigt, Florian organization: Department of Cardiology, Krankenhaus Porz, Urbacher Weg, Cologne, Germany – sequence: 2 givenname: Lars surname: Eichhorn fullname: Eichhorn, Lars organization: Department of Anesthesiology, University Hospital Bonn, Bonn, Germany – sequence: 3 givenname: Thomas surname: Beiert fullname: Beiert, Thomas organization: Department of Cardiology, University Hospital Bonn, Bonn, Germany – sequence: 4 givenname: Vincent surname: Knappe fullname: Knappe, Vincent organization: Department of Cardiology, University Hospital Bonn, Bonn, Germany – sequence: 5 givenname: Tobias surname: Radecke fullname: Radecke, Tobias organization: Department of Cardiology, University Hospital Essen, Hufelandstraße, Essen, Germany – sequence: 6 givenname: Martin surname: Steinmetz fullname: Steinmetz, Martin organization: Department of Cardiology, University Hospital Essen, Hufelandstraße, Essen, Germany – sequence: 7 givenname: Georg surname: Nickenig fullname: Nickenig, Georg organization: Department of Cardiology, University Hospital Bonn, Bonn, Germany – sequence: 8 givenname: Viktoriya surname: Peeva fullname: Peeva, Viktoriya organization: Department of Epileptology, University Hospital of Bonn, Bonn, Germany – sequence: 9 givenname: Alexei P surname: Kudin fullname: Kudin, Alexei P organization: Department of Epileptology, University Hospital of Bonn, Bonn, Germany – sequence: 10 givenname: Wolfram S surname: Kunz fullname: Kunz, Wolfram S organization: Department of Epileptology, University Hospital of Bonn, Bonn, Germany – sequence: 11 givenname: Carolin surname: Berwanger fullname: Berwanger, Carolin organization: Institute of Aerospace Medicine, German Aerospace Center (DLR), Linder Höhe, Cologne, Germany – sequence: 12 givenname: Lisa surname: Kamm fullname: Kamm, Lisa organization: Institute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany – sequence: 13 givenname: Dorothea surname: Schultheis fullname: Schultheis, Dorothea organization: Institute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany – sequence: 14 givenname: Ursula surname: Schlötzer-Schrehardt fullname: Schlötzer-Schrehardt, Ursula organization: Department of Opthalmology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany – sequence: 15 givenname: Christoph S surname: Clemen fullname: Clemen, Christoph S organization: Center for Physiology and Pathophysiology, Institute of Vegetative Physiology, Medical Faculty, University of Cologne, Cologne, Germany – sequence: 16 givenname: Rolf surname: Schröder fullname: Schröder, Rolf organization: Institute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Schwabachanlage, Erlangen, Germany – sequence: 17 givenname: Jan W surname: Schrickel fullname: Schrickel, Jan W organization: Department of Cardiology, University Hospital Bonn, Bonn, Germany |
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| Snippet | Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV blocks. We... Background Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV... BACKGROUNDMutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV... BACKGROUND:Mutations in the human desmin gene (DES) cause autosomal-dominant and -recessive cardiomyopathies, leading to heart failure, arrhythmias, and AV... |
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| SubjectTerms | Analysis Arrhythmia Biology and Life Sciences Echocardiography Genes Heart failure Hypertension Medicine and Health Sciences Myocardial diseases Research and Analysis Methods Tachycardia |
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| Title | Heart failure after pressure overload in autosomal-dominant desminopathies: Lessons from heterozygous DES-p.R349P knock-in mice |
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