Progeria

I think progeria is a fascinating disease. Jonathan   Hutchinson   and Hastings Gilford, both English surgeons, described the symptoms of progeria independently in 1886. Thus, an alternate name for progeria is Hutchinson-Gilford syndrome. Hutchinson invented the shorter term progeria, a combination of Greek words meaning “prematurely old” in 1904. Since then, in excess of 100 cases of progeria have been recorded worldwide. Scientists have hypothesized that a genetic mutation during conception is the cause of the disease, but they are not sure how to cure the disease.
Individuals affected by progeria have symptoms appearing to correspond to natural old age in childhood and early teens; progeria- prematurely ages children. Their skin becomes dry, their muscle atrophy and they suffer from atherosclerosis and other diseases normally associated with older people. There are other symptoms of progeria besides an aged phenotype: “dwarfism, a pinched nose, small face and small jaw (micrognathia), delayed tooth formation and aged-looking skin. Intelligence is normal or above average. The voice is thin and high-pitched. Sexual maturation does not occur.
Richard Allsopp and his coworkers found in 1992 that the cells of children with progeria have shorter telomeres than the cells of unaffected individuals of comparable ages. Teomeres are repeats of the sequence TTAGGG on the ends of a chromosome. Bits of them are removed every time the chromosome goes through mitosis, limiting the number of times a normal cell may divide. Once the amount of telomeres on the tip of any chromosome reaches a certain paucity, the chromosome will not divide any more times. A shorter than normal telomere entails less times a cell may divide and therefore a faster than normal aging process. Although the victims of progeria have shorter telomeres on the tips of their chromosomes in every cell, not all early-aging diseases have telomeres of similar length: “in other aging-genetics disorders, such as...