Researchers studying Hutchison-Gilford progeria syndrome have discovered protein functions that could help healthy people live longer lives. Hutchison-Gilford progeria syndrome (HGPS) is a rare genetic disorder that causes its victims to age must faster than normal. It manifests itself during the first few months of a child’s life, with additional symptoms becoming readily apparent at between 18 and 24 months. Those who suffer from HGPS experience symptoms that generally only affect the elderly, such as degeneration of muscle and joints, joint stiffness, hip dislocations, etc. Mental development and motor skills typically remain normal.
HGPS is thought to be caused by a rare mutation in a protein within the body known as LMNA. It is estimated to occur in 1 in 4 million births. There are currently 100 known cases in the world, with 140 cases having been reported in medical history. Victims of HGPS rarely live to child-bearing age, as most die before their 13th birthday. Therefore the disease is rarely transmitted from parent to child, occurring in the overwhelming majority of cases as a one-off mutation.
Researchers looking into protein synthesis among HGPS sufferers found that it wasn’t just LMNA that was affected. Other proteins in the body showed signs of overactive protein synthesis, the process by which the body’s cells generate new proteins. Since research in mice and roundworms suggests that slowing down protein synthesis might lead to lengthened lifespans, this latest research might point to accelerated protein synthesis as a source of aging, not just among HGPS patients but also among the general population. Treatments that could slow down the process of protein synthesis could potentially benefit those who suffer from HGPS as well as slow down the aging process among the healthy.