A Rare Connection: Fasciculoventricular Pathway in PRKAG2 Disease
Autor: | Elijah R. Behr, Malini Govindan, David E. Ward |
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Rok vydání: | 2010 |
Předmět: |
Genetics
Mutation Heart Ventricles Protein subunit Disease AMP-Activated Protein Kinases Middle Aged Biology medicine.disease_cause Rare Diseases Heart Conduction System Physiology (medical) Cardiac hypertrophy Ventricular Fibrillation Cancer research medicine Humans Female Electrical conduction system of the heart Cardiology and Cardiovascular Medicine Protein kinase A Mahaim fiber Gene |
Zdroj: | Journal of Cardiovascular Electrophysiology. 21:329-332 |
ISSN: | 1540-8167 1045-3873 |
DOI: | 10.1111/j.1540-8167.2009.01578.x |
Popis: | Fasciculoventricular Pathway in PRKAG2 Disease. Mutations in the PRKAG2 gene that regulates the gamma 2 subunit of the AMP-dependent protein kinase A have been associated with the development of AV accessory pathways, cardiac hypertrophy, and conduction system abnormalities. The accessory pathways described in PRKAG2 disease have mostly been AV bypass tracts, as mutations in the PRKAG2 gene disrupt the normal AV junction development. There have also been a few reports of nodoventricular tracts associated with PRKAG2 mutations, as these tracts also involve the AV junction. We describe a case of a fasciculoventricular pathway with PRKAG2 mutation suggesting a more widespread involvement of the PRKAG2 gene in the development of the cardiac conduction system. (J Cardiovasc Electrophysiol, Vol. 21, pp. 329–332, March 2010) |
Databáze: | OpenAIRE |
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