replication stress induces senescence in progeria

Featured Image

Why is it important?

The aberrant lamin A protein (progerin) that is made in the cells of children with Hutchinson-Gilford Progeria Syndrome interferes with DNA replication by sequestering a key protein that is required for DNA synthesis - PCNA. As a results, the rate of DNA replication fork progression is decreased and the cells exhibit replication stress resulting in DNA strand breaks and the activation of the p53 protein leading to premature cellular senescence.