Gap junction remodeling and cardiac arrhythmogenesis in a murine model of oculodentodigital dysplasia
Gap junction channels are required for normal cardiac impulse propagation, and gap junction remodeling is associated with enhanced arrhythmic risk. Oculodentodigital dysplasia (ODDD) is a multisystem syndrome due to mutations in the connexin43 (Cx43) gap junction channel gene. To determine the effec...
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Published in | Proceedings of the National Academy of Sciences - PNAS Vol. 104; no. 51; pp. 20512 - 20516 |
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Main Authors | , , , , , , , , , |
Format | Journal Article |
Language | English |
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United States
National Academy of Sciences
18.12.2007
National Acad Sciences |
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Abstract | Gap junction channels are required for normal cardiac impulse propagation, and gap junction remodeling is associated with enhanced arrhythmic risk. Oculodentodigital dysplasia (ODDD) is a multisystem syndrome due to mutations in the connexin43 (Cx43) gap junction channel gene. To determine the effects of a human connexin channelopathy on cardiac electrophysiology and arrhythmogenesis, we generated a murine model of ODDD by introducing the disease-causing I130T mutant allele into the mouse genome. Cx43 abundance was markedly reduced in mutant hearts with preferential loss of phosphorylated forms that interfered with trafficking and assembly of gap junctions in the junctional membrane. Dual whole-cell patch-clamp studies showed significantly lower junctional conductance between neonatal cell pairs from mutant hearts, and optical mapping of isolated-perfused hearts with voltage-sensitive dyes demonstrated significant slowing of conduction velocity. Programmed electrical stimulation revealed a markedly increased susceptibility to spontaneous and inducible ventricular tachyarrhythmias. In summary, our data demonstrate that the I130T mutation interferes with Cx43 posttranslational processing, resulting in diminished cell-cell coupling, slowing of impulse propagation, and a proarrhythmic substrate. |
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AbstractList | Gap junction channels are required for normal cardiac impulse propagation, and gap junction remodeling is associated with enhanced arrhythmic risk. Oculodentodigital dysplasia (ODDD) is a multisystem syndrome due to mutations in the connexin43 (Cx43) gap junction channel gene. To determine the effects of a human connexin channelopathy on cardiac electrophysiology and arrhythmogenesis, we generated a murine model of ODDD by introducing the disease-causing I130T mutant allele into the mouse genome. Cx43 abundance was markedly reduced in mutant hearts with preferential loss of phosphorylated forms that interfered with trafficking and assembly of gap junctions in the junctional membrane. Dual whole-cell patch-clamp studies showed significantly lower junctional conductance between neonatal cell pairs from mutant hearts, and optical mapping of isolated-perfused hearts with voltage-sensitive dyes demonstrated significant slowing of conduction velocity. Programmed electrical stimulation revealed a markedly increased susceptibility to spontaneous and inducible ventricular tachyarrhythmias. In summary, our data demonstrate that the I130T mutation interferes with Cx43 posttranslational processing, resulting in diminished cell-cell coupling, slowing of impulse propagation, and a proarrhythmic substrate. [PUBLICATION ABSTRACT] Gap junction channels are required for normal cardiac impulse propagation, and gap junction remodeling is associated with enhanced arrhythmic risk. Oculodentodigital dysplasia (ODDD) is a multisystem syndrome due to mutations in the connexin43 ( Cx43 ) gap junction channel gene. To determine the effects of a human connexin channelopathy on cardiac electrophysiology and arrhythmogenesis, we generated a murine model of ODDD by introducing the disease-causing I130T mutant allele into the mouse genome. Cx43 abundance was markedly reduced in mutant hearts with preferential loss of phosphorylated forms that interfered with trafficking and assembly of gap junctions in the junctional membrane. Dual whole-cell patch–clamp studies showed significantly lower junctional conductance between neonatal cell pairs from mutant hearts, and optical mapping of isolated-perfused hearts with voltage-sensitive dyes demonstrated significant slowing of conduction velocity. Programmed electrical stimulation revealed a markedly increased susceptibility to spontaneous and inducible ventricular tachyarrhythmias. In summary, our data demonstrate that the I130T mutation interferes with Cx43 posttranslational processing, resulting in diminished cell–cell coupling, slowing of impulse propagation, and a proarrhythmic substrate. Gap junction channels are required for normal cardiac impulse propagation, and gap junction remodeling is associated with enhanced arrhythmic risk. Oculodentodigital dysplasia (ODDD) is a multisystem syndrome due to mutations in the connexin43 ( Cx43 ) gap junction channel gene. To determine the effects of a human connexin channelopathy on cardiac electrophysiology and arrhythmogenesis, we generated a murine model of ODDD by introducing the disease-causing I130T mutant allele into the mouse genome. Cx43 abundance was markedly reduced in mutant hearts with preferential loss of phosphorylated forms that interfered with trafficking and assembly of gap junctions in the junctional membrane. Dual whole-cell patch–clamp studies showed significantly lower junctional conductance between neonatal cell pairs from mutant hearts, and optical mapping of isolated-perfused hearts with voltage-sensitive dyes demonstrated significant slowing of conduction velocity. Programmed electrical stimulation revealed a markedly increased susceptibility to spontaneous and inducible ventricular tachyarrhythmias. In summary, our data demonstrate that the I130T mutation interferes with Cx43 posttranslational processing, resulting in diminished cell–cell coupling, slowing of impulse propagation, and a proarrhythmic substrate. arrhythmia connexin43 transgenic channel mouse Gap junction channels are required for normal cardiac impulse propagation, and gap junction remodeling is associated with enhanced arrhythmic risk. Oculodentodigital dysplasia (ODDD) is a multisystem syndrome due to mutations in the connexin43 (Cx43) gap junction channel gene. To determine the effects of a human connexin channelopathy on cardiac electrophysiology and arrhythmogenesis, we generated a murine model of ODDD by introducing the disease-causing I130T mutant allele into the mouse genome. Cx43 abundance was markedly reduced in mutant hearts with preferential loss of phosphorylated forms that interfered with trafficking and assembly of gap junctions in the junctional membrane. Dual whole-cell patch-clamp studies showed significantly lower junctional conductance between neonatal cell pairs from mutant hearts, and optical mapping of isolated-perfused hearts with voltage-sensitive dyes demonstrated significant slowing of conduction velocity. Programmed electrical stimulation revealed a markedly increased susceptibility to spontaneous and inducible ventricular tachyarrhythmias. In summary, our data demonstrate that the I130T mutation interferes with Cx43 posttranslational processing, resulting in diminished cell-cell coupling, slowing of impulse propagation, and a proarrhythmic substrate. |
Author | Qu, Jiaxiang Sandeep, Nefthi Suadicani, Sylvia O Garcia, Luis Wang, Zhiyong Zhang, Jie Fishman, Glenn I Kalcheva, Nellie Spray, David C Lampe, Paul D |
Author_xml | – sequence: 1 fullname: Kalcheva, Nellie – sequence: 2 fullname: Qu, Jiaxiang – sequence: 3 fullname: Sandeep, Nefthi – sequence: 4 fullname: Garcia, Luis – sequence: 5 fullname: Zhang, Jie – sequence: 6 fullname: Wang, Zhiyong – sequence: 7 fullname: Lampe, Paul D – sequence: 8 fullname: Suadicani, Sylvia O – sequence: 9 fullname: Spray, David C – sequence: 10 fullname: Fishman, Glenn I |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/18077386$$D View this record in MEDLINE/PubMed |
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Notes | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 Edited by Charles S. Peskin, New York University, New York, NY, and approved November 7, 2007 Author contributions: G.I.F. designed research; N.K., J.Q., N.S., L.G., J.Z., Z.W., P.D.L., S.O.S., and D.C.S. performed research; P.D.L. contributed new reagents/analytic tools; J.Q., N.S., L.G., S.O.S., D.C.S., and G.I.F. analyzed data; and N.K. and G.I.F. wrote the paper. |
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SubjectTerms | Abnormalities, Multiple - genetics Animals Antibodies Arrhythmias, Cardiac - genetics Arrhythmias, Cardiac - metabolism Biological Sciences Cardiac arrhythmia Cardiac Electrophysiology Conduction Connexin 43 - deficiency Connexin 43 - genetics Craniofacial Abnormalities - genetics Disease Models, Animal Dyes Dysrhythmias Electric potential Gap junctions Gap Junctions - genetics Gap Junctions - metabolism Genetic mutation Genomics Heart - physiopathology Heart Conduction System - physiopathology Isoquinolines - analysis Isoquinolines - metabolism Lower Extremity Deformities, Congenital - genetics Mice Mice, Knockout Mutation Myocytes, Cardiac - pathology Phosphorylation Renovations Rodents Syndrome |
Title | Gap junction remodeling and cardiac arrhythmogenesis in a murine model of oculodentodigital dysplasia |
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