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www.ijfstonline.org PROGERIA By:  Prithivi Logathasan,(B-Pharm III Year),Vels University HGPS (Hutchinson-Gilford Progeria Syndrome) or Progeria is an extremely rare, fatal genetic condition....

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Home Newsletters NL-1-2014-LETTER-4 -PROGERIA-By-Prithivi Logathasan
NL-1-2014-LETTER-4 -PROGERIA-By-Prithivi Logathasan PDF Print E-mail

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PROGERIA

By:  Prithivi Logathasan,(B-Pharm III Year),Vels University

HGPS (Hutchinson-Gilford Progeria Syndrome) or Progeria is an extremely rare, fatal genetic condition. The word Progeria comes from the Greek progeros meaning 'prematurely old'. The Greek word pro means 'before', while the word geras means 'old age'. Progeria affects children and gives them an appearance of accelerated aging. The classic type of Progeria (there are different forms) is Hutchinson-Gilford Progeria Syndrome (HGPS). Progeria was first described in an academic journal by Dr. Jonathan Hutchinson in 1886, and Dr. Hastings Gilford in 1897 - both in England.

Signs and symptoms:

Progeria affects one in every four million to eight million births; there are about 50 cases currently recognized worldwide with 10 to 12 in the U.S. Children with progeria usually develop the first symptoms during their first few months. The earliest symptoms may include a failure to thrive and a localized scleroderma-like skin condition. As a child ages past infancy, additional conditions become apparent usually around 18–24 months. Limited growth, full-body alopecia, and a distinctive appearance (a small face with a shallow recessed jaw, and a pinched nose) are all characteristics of progeria. Signs and symptoms of this progressive disease tend to get worse as the child ages. Later, the condition causes wrinkled skin, atherosclerosis, kidney failure, loss of eyesight, hair loss, and cardiovascular problems. Scleroderma, a hardening and tightening of the skin on trunk and extremities of the body, is prevalent. People diagnosed with this disorder usually have small, fragile bodies, like those of elderly people. The face is usually wrinkled, with a larger head in relation to the body, a narrow face and a beak nose. Prominent scalp veins are noticeable (made more obvious by alopecia), as well as prominent eyes. Musculoskeletal degeneration causes loss of body fat and muscle, stiff joints, hip dislocations, and other symptoms generally absent in the non-elderly population. Individuals do usually retain normal mental and motor development. about 90 percent of Progeria patients die by age 13 from fatal heart attacks or strokes.

Cause:

Researchers say that each progeria case arises randomly due to a single letter change in one gene of the child's DNA. The mutation—believed to occur in the father’s sperm before conception—results in the production of a toxic protein that attaches to and distorts the nucleus (the cell's command center containing its genetic material). Although cells normally multiply during growth and development, the misshapen nucleus cannot divide properly, ultimately damaging cells and accelerating the aging process.

Cardiovascular disease in these young patients develops as vulnerable cells lining the interior of major arteries (vessels that carry blood away from the heart) accumulate the toxic protein and die. This causes the arteries to stiffen and crack, leading to plaque buildup that blocks blood flow.

Research on progeria medication and latest drug discovery:

Low-dose aspirin is often used to help prevent heart attacks, but some children even undergo bypass surgery or angioplasty (dilation of the arteries) to slow the disease.   Genetically engineered mice carrying the progeria mutation were used to test the effectiveness of farnesyltransferase inhibitors (FTIs) in combating progeria-related cardiovascular disease. FTIs restore the shape of the nucleus, thereby saving cells from premature destruction by preventing the toxic protein from attaching to the command hub.

 

Researchers found that FTIs not only prevented cardiovascular damage in young mice, but also reversed the disease in older animals treated after the onset of arterial damage. "We were amazed that [the drug] worked so well," says Francis Collins, who led the research team that identified the progeria gene mutation in 2003. Beyond progeria, these results have the potential to benefit all patients with cardiovascular disease. Researchers have discovered that the toxic protein responsible for progeria is actually produced at low levels in all humans, possibly accumulating as we age. Thus, by studying these rare children, we can further our understanding of a major mechanism of human aging—and perhaps, find new ways to slow the process.

 

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