Causes of Hutchinson-Gilford Syndrome (Progeria)
- Progeria is a rare condition that is very interesting due to the fact that its symptoms strongly resemble normal human aging, but occur in young children. This disease is not usually passed down from one generation to the next. Progeria is hardly seen in more than one child in a family.
-The cause of progeria has to do with the differences that are evident in the formation of a regular cell and a progeria cell. Researchers have discovered a single gene mutation responsible for Hutchinson-Gilford progeria syndrome. The gene is known as lamin A (LMNA), which makes a protein necessary for holding the center (nucleus) of a cell together. Researchers believe the genetic mutation renders cells unstable, which appears to lead to progeria's characteristic aging process.Unlike many genetic mutations, Hutchinson-Gilford progeria isn't passed down in families. Rather, the gene change is a chance occurrence that researchers believe affects a single sperm or egg just before conception. Neither parent is a carrier, so the mutations in the children's genes are new. Below is an image of the formation of a Normal cell and a Progeria cell.