Topics: Progeria, Senescence, Gerontology Pages: 4 (1594 words) Published: November 3, 2007
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 not reproduce Livnech 1). There is evidence that shows the gene for Progeria may be on chromosome 1. It is believed that gene is passed on in autosomal dominant inheritance. Autosomal dominant inheritance means that either affected parent has a 50% chance of passing it on. The occurrence of Progeria is sporadic and rare. Studies show frequency may be related to a high difference in the age of parents. There have been 100 cases reported where that is the case (Progeria 1). There is a 1/5:1 male to female ratio when it comes to the statistics of those with Progeria (Livnech 2). It is not believed to run in families. Researchers believe that the disorder stems from a chance anomaly in the sperm or egg before conception (Haran 1). When a child has Progeria...
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