Channelopathies of voltage-gated L-type Cav1.3/α1D and T-type Cav3.1/α1G Ca2+ channels in dysfunction of heart automaticity

Autor: Matteo E. Mangoni, Angelo G. Torrente, P. Mesirca, Isabelle Bidaud
Přispěvatelé: Guerineau, Nathalie C., Institut de Génomique Fonctionnelle (IGF), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), LabEx Ion Channel Science and Therapeutics, Université de Montpellier (UM)-Université Montpellier 1 (UM1)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Montpellier 2 - Sciences et Techniques (UM2)-Centre National de la Recherche Scientifique (CNRS)
Rok vydání: 2020
Předmět:
0301 basic medicine
[SDV.MHEP.PHY] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO]
Physiology
Heart block
Sinus node dysfunction
Clinical Biochemistry
Population
Automaticity
sinus node
Cav1.3
03 medical and health sciences
0302 clinical medicine
[SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system
Ca 2+ channels
Physiology (medical)
[SDV.MHEP.PHY]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO]
medicine
Myocyte
education
Ion channel
education.field_of_study
biology
Voltage-gated ion channel
business.industry
medicine.disease
[SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system
3. Good health
030104 developmental biology
biology.protein
Ca2+ channels
Electrical conduction system of the heart
business
Neuroscience
Cav3.1
030217 neurology & neurosurgery
Zdroj: Pflügers Archiv European Journal of Physiology
Pflügers Archiv European Journal of Physiology, 2020, 472 (7), pp.817-830. ⟨10.1007/s00424-020-02421-1⟩
Pflügers Archiv European Journal of Physiology, Springer Verlag, 2020, 472 (7), pp.817-830. ⟨10.1007/s00424-020-02421-1⟩
ISSN: 1432-2013
0031-6768
Popis: International audience; The heart automaticity is a fundamental physiological function in vertebrates. The cardiac impulse is generated in the sinus node by a specialized population of spontaneously active myocytes known as "pacemaker cells." Failure in generating or conducting spontaneous activity induces dysfunction in cardiac automaticity. Several families of ion channels are involved in the generation and regulation of the heart automaticity. Among those, voltage-gated L-type Cav1.3 (α1D) and T-type Cav3.1 (α1G) Ca2+ channels play important roles in the spontaneous activity of pacemaker cells. Ca2+ channel channelopathies specifically affecting cardiac automaticity are considered rare. Recent research on familial disease has identified mutations in the Cav1.3-encoding CACNA1D gene that underlie congenital sinus node dysfunction and deafness (OMIM # 614896). In addition, both Cav1.3 and Cav3.1 channels have been identified as pathophysiological targets of sinus node dysfunction and heart block, caused by congenital autoimmune disease of the cardiac conduction system. The discovery of channelopathies linked to Cav1.3 and Cav3.1 channels underscores the importance of Ca2+ channels in the generation and regulation of heart's automaticity.
Databáze: OpenAIRE
Externí odkaz: