Epigenetic Involvement in Hutchinson-Gilford Progeria Syndrome: A Mini-Review

  title={Epigenetic Involvement in Hutchinson-Gilford Progeria Syndrome: A Mini-Review},
  author={W. Arancio and G. Pizzolanti and S. Genovese and M. Pitrone and C. Giordano},
  pages={197 - 203}
  • W. Arancio, G. Pizzolanti, +2 authors C. Giordano
  • Published 2014
  • Biology, Medicine
  • Gerontology
  • Hutchinson-Gilford progeria syndrome (HGPS) is a rare human genetic disease that leads to a severe premature ageing phenotype, caused by mutations in the LMNA gene. The LMNA gene codes for lamin-A and lamin-C proteins, which are structural components of the nuclear lamina. HGPS is usually caused by a de novo C1824T mutation that leads to the accumulation of a dominant negative form of lamin-A called progerin. Progerin also accumulates physiologically in normal ageing cells as a rare splicing… CONTINUE READING
    44 Citations
    Hutchinson–Gilford progeria syndrome as a model for vascular aging
    • 27
    BK channel overexpression on plasma membrane of fibroblasts from Hutchinson-Gilford progeria syndrome
    • 2
    • PDF
    Genetic Variations: Heroes or Villains
    • 1


    Progerin and telomere dysfunction collaborate to trigger cellular senescence in normal human fibroblasts.
    • 233
    • PDF
    A ceRNA analysis on LMNA gene focusing on the Hutchinson-Gilford progeria syndrome
    • 16
    A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease.
    • 13
    Mutant nuclear lamin A leads to progressive alterations of epigenetic control in premature aging.
    • 603
    • PDF