Progeria, the Premature Fatal Aging Disorder in Children, May Be Able to Be Reversed Through Farnesyltransferase Inhibitors Treatments

Progeria, the premature fatal aging disorder in children, may be able to be reversed through Farnesyltransferase inhibitors (FTI) treatments.

Formally known as Hutchinson - Gilford syndrome, Progeria is a genetic disorder that affects 1 in every 8 million babies born. The disorder is known for its unusual appearance of premature aging in children. Progeria was first discovered when it showed up in a child in 1886 by Dr. Hutchinson. The second case was later discovered by Gilford a year later. In 1889 Badame named the disorder Progeria, derived from a Greek word meaning “old age” (Livneh 1). The suspected cause of Progeria is a mutation in Lamin A/C gene that leads to abnormality in control of the cell division and in the formation and reconstruction of collagen and extra cellular matrix (Manrai 2). It is still unclear where Progeria comes from. Some researchers think an autosomal recessive transmission while others think the mode of inheritance is autosomal dominant mutation. Though those ideas are in place regarding where Progeria came from it is still undecided how Progeria is inherited. It is difficult for researchers to confirm the mode of genetic transmission because Progeria patients do