Progeria, which is Greek for “prematurely old,” is a rare genetic disorder in which young children appear to age rapidly and is caused by a mutation in the LMNA gene. Approximately 1 in 8 million children are born with Progeria worldwide. The progression of this disease is comparable to aging at a rate six to eight times faster than normal aging. Symptoms of this condition are present in early childhood and include failure to thrive and skin conditions. A distinctive appearance then emerges, including a small face, a flat nose, hair loss, and a frail body. Over time, this disease causes cardiovascular problems, and patients usually die of heart disease in their early teens. Say no to plagiarism. Get a tailor-made essay on "Why Violent Video Games Shouldn't Be Banned"? Get an original essayProgeria is caused by a mutation in the nuclear lamina. The laminae form the nuclear lamina, which is the inner side of the nuclear envelope and peripheral chromatin. There are four major lamin proteins: lamin A, C, B1, and B2. These proteins help in many nuclear processes such as chromatin structure, regulation of gene expression, apoptosis, cell cycle regulation, nuclear migration, and protein degradation. Because these proteins affect so many different cellular functions, mutations can cause widespread problems. Problems arise in a mutation in the LMNA gene. This gene, located on the first chromosome, usually produces Lamin. A de novo point mutation (i.e., a mutation that occurred spontaneously and was not passed down) replaces cytosine with thymine at position 1824 of the LMNA gene. The mutation creates a DNA splice site where there usually isn't one. This creates a problem with DNA transcription and changes exon 11. The change in DNA information ultimately produces a mutant protein, known as Progerin, that becomes lipidated or acquires hydrophobic molecules. This protein is then abnormally incorporated into the nuclear lamina, and the nucleus becomes unstable with the addition of this malformed protein. The collection of faulty proteins can lead to mechanical problems, overgrowth or loss of lamina, and DNA damage. By changing a nucleotide in the genetic code, it is not possible to produce an essential protein, altering the organism drastically. Ongoing research into the causes and treatments of Progeria may not only help people with this disease but also those who suffer from closely related conditions such as Emery -Dreifuss Muscular Dystrophy and Restrictive Dermopathy. Another benefit of this research is also the acquisition of information on cardiovascular diseases and normal aging. In 2003, Swedish scientists discovered the mutant protein, Progerin, in both Progeria patients and normal cells, but at varying levels. As normal cells aged, progerin accumulated in these cells. This evidence shows a direct link between Progeria, this mutated protein, and old age. The study of Progeria can provide us with valuable information on the natural aging process. One hope for the treatment of Progeria is the inhibition of protein farinasylation or the lipidation process. Through the use offarnesyltransferase inhibitors (FTIs) it is possible to block damage to the nuclear lamina. These cells may also be able to be repaired through this treatment, which is a big breakthrough since patients are usually not diagnosed with Progeria until the damage has already begun. It seems.