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Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations

Autor
Riveras, J.; Calvo, C.; Llach, A.; Gúzman, G.; Caballero, R.; González, C.; Jiménez, L.; Guadix, J.; Osorio, F.; López, C.; Herraiz, A.; Cabello, N.; Vallmitjana, A.; Benitez, R.; Gordon, L.
Tipus d'activitat
Article en revista
Revista
PNAS - National Academy of Sciences. Proceedings (Online)
Data de publicació
2016-11-15
Volum
113
Número
46
Pàgina inicial
E7250
Pàgina final
E7259
DOI
https://doi.org/10.1073/pnas.1603754113 Obrir en finestra nova
Repositori
http://hdl.handle.net/2117/100094 Obrir en finestra nova
URL
http://www.pnas.org/content/113/46/E7250 Obrir en finestra nova
Resum
Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disease caused by defective prelamin A processing, leading to nuclear lamina alterations, severe cardiovascular pathology, and premature death. Prelamin A alterations also occur in physiological aging. It remains unknown how defective prelamin A processing affects the cardiac rhythm. We show age-dependent cardiac repolarization abnormalities in HGPS patients that are also present in the Zmpste24-/- mouse model of HGPS. Challenge of ...
Citació
Riveras, J., Calvo, C., Llach, A., Gúzman, G., Caballero, R., González, C., Jiménez, L., Guadix, J., Osorio, F., López, C., Herraiz, A., Cabello, N., Vallmitjana, A., Benitez, R., Gordon, L. Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations. "PNAS - National Academy of Sciences. Proceedings (Online)", 15 Novembre 2016, vol. 113, núm. 46, p. E7250-E7259.
Paraules clau
Hutchinson–gilford Progeria Syndrome, Progerin, Prelamin A, Connexin43, Calcium Handling
Grup de recerca
CREB - Centre de Recerca en Enginyeria Biomedica
SISBIO - Senyals i Sistemes Biomèdics

Participants

  • Riveras Torres, Jose  (autor)
  • Calvo, Conrado J.  (autor)
  • Llach, Anna  (autor)
  • Gúzman Martínez, Gabriela  (autor)
  • Caballero, Ricardo  (autor)
  • González Gómez, Cristina  (autor)
  • Jiménez Borreguero, Luís J.  (autor)
  • Guadix, Juan A.  (autor)
  • Osorio, Fernando G.  (autor)
  • López Otín, Carlos  (autor)
  • Herraiz Martínez, Adela  (autor)
  • Cabello, Nuria  (autor)
  • Vallmitjana Lees, Alexander  (autor)
  • Benitez Iglesias, Raul  (autor)
  • Gordon, Leslie B.  (autor)

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