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

Author
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.
Type of activity
Journal article
Journal
PNAS - National Academy of Sciences. Proceedings (Online)
Date of publication
2016-11-15
Volume
113
Number
46
First page
E7250
Last page
E7259
DOI
https://doi.org/10.1073/pnas.1603754113 Open in new window
Repository
http://hdl.handle.net/2117/100094 Open in new window
URL
http://www.pnas.org/content/113/46/E7250 Open in new window
Abstract
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 ...
Citation
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.
Keywords
Hutchinson–Gilford progeria syndrome, calcium handling, connexin43, prelamin A, progerin
Group of research
ANCORA - Anàlisi i control del ritme cardíac
B2SLab - Bioinformatics and Biomedical Signals Laboratory
CREB - Biomedical Engineering Research Centre

Participants

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

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