Expression data from progeroid smooth muscle cells

Hutchinson-Gilford Progeria syndrome (HGPS or Progeria) is a rare and fatal genetic condition, characterized by premature aging symptoms in children and it affects approximately 1 in 4-8 million newborns. Individuals with HGPS appear to show aging-related phenotypes at a much faster rate than normal, leaving young children with the appearance and health conditions of an aged individual. This syndrome causes changes in various organs and systems such as the skin, skeleton, hair, body fat and cardiovascular system. One of the hallmarks of the disease is the loss of SMCs in the medial layer of large arteries with replacement by collagen and extracellular matrix, and in many cases calcification. We used microarrays to detail the global programme of gene expression underlying smooth muscle cell loss and identified distinct classes of up- and down-regulated genes during this process. The analyses were performed on progeria smooth muscle cells derived from progeria iPSCs and healthy (N) smooth muscle cells derived from iPSCs at static conditions or cultured for 4 days in arterial flow conditions. In both cases total RNA from cells was extracted and gene expression was evaluated by a whole human genome microarray Human Gene 2.1 ST Array Strip from Affymetrix.