Modeling of lamin A/C mutation premature cardiac aging using patient-specific induced pluripotent stem cells
Aging2012Vol. 4(11), pp. 803–822
Citations Over TimeTop 10% of 2012 papers
Chung‐Wah Siu, Yee-Ki Lee, Jenny Chung-Yee Ho, Wing‐Hon Lai, Yau-Chi Chan, Kwong‐Man Ng, Lai‐Yung Wong, Ka‐Wing Au, Yee‐Man Lau, Jinqiu Zhang, Kenneth Lay, Alan Colman, Hung‐Fat Tse
Abstract
LMNA‐related DCM was modeled in‐vitro using patient‐specific iPSC‐CMs. Our results demonstrated that haploinsufficiency due to R225X LMNA non‐sense mutation was associated with accelerated nuclear senescence and apoptosis of iPSC‐ CMs under electrical stimulation, which can be significantly attenuated by therapeutic blockade of stress‐related ERK1/2 pathway.
Related Papers
- → Nuclear Lamins: Laminopathies and Their Role in Premature Ageing(2006)566 cited
- → Hutchinson–Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody(2006)195 cited
- → Molecular insights into the premature aging disease progeria(2016)109 cited
- → DNA damage responses caused by Zmpste24 deficiency and lamin A mutation in premature ageing(2006)
- The multiple roles of A-type lamins in cellular aging, cell cycle progression and the DNA damage response(2011)