DNA repair defects and genome instability in Hutchinson-Gilford Progeria Syndrome.

@article{Gonzalo2015DNARD,
  title={DNA repair defects and genome instability in Hutchinson-Gilford Progeria Syndrome.},
  author={S Gonzalo and Ray Kreienkamp},
  journal={Current opinion in cell biology},
  year={2015},
  volume={34},
  pages={75-83}
}
The integrity of the nuclear lamina has emerged as an important factor in the maintenance of genome stability. In particular, mutations in the LMNA gene, encoding A-type lamins (lamin A/C), alter nuclear morphology and function, and cause genomic instability. LMNA gene mutations are associated with a variety of degenerative diseases and devastating premature aging syndromes such as Hutchinson-Gilford Progeria Syndrome (HGPS) and Restrictive Dermopathy (RD). HGPS is a severe laminopathy, with… CONTINUE READING
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