Hutchinson-Gilford progeria syndrome (HGPS), or simply progeria, is a rare genetic condition that presents the superficial appearance of greatly accelerated aging. It isn’t in fact accelerated aging, but rather one specific form of molecular damage run amok, causing severe and increasing dysfunction in near all cells. Normal aging is a collection of many varied forms of molecular damage that eventually cause severe and increasing dysfunction in near all cells. The consequences of a failure of any given population of cells or an organ to function correctly can appear superficially similar even if the causes are not the same, but as soon as one digs in to the details, the different mechanisms become evidence and significant.
Soon after the turn of the century, the cause of progeria was identified: a mutation in the LMNA gene that gives rise to a broken protein now called progerin. The normal, unbroken form of this protein, lamin A, is a vital part of the internal structure of a cell, and once once that structural support falters, all of the other finely balanced mechanisms begin to fail as well. Soon thereafter, it was discovered that progerin can also be
Article originally posted at