The Cause
The exact cause of "Progeria" is not known. At the moment it
is believed due to a single abnormal (mutant), as-yet unidentified, gene.
It is not known yet whether more than one gene is involved. Because Progeria
within the families appears only sporadically and rarely, and because
children with Progeria are unable to reproduce, family studies to identify
the cause of the disease are not possible. Because neither parent carries
or expresses the mutation, each case is believed to represent a sporadic
new mutation, which happens at the time of conception. A prenatal diagnosis
is also not feasible.
The science sees the "key to immortality" in Progeria. One theory
is that a altered DNA section (shortened Telomere) causes the accelerated
aging process and Progeria symptoms. Researchers who believe in this connection
have found that end sections of the chromosomes (Telomeres) contain the
key to extended life. Shortly after birth, the Telomere shrinks. With
every cell division approximately 50 components of the DNA are used. The
elixir of the cell is used up after 80 to 100 cell divisions. Following
this theory, this limits our life to 120 years maximum. Without Telomeres,
cells die. If the Telomeres are worn out, there is nothing else to prevent
the body from decaying. According to the so-called "free radical"
theory, destroyed cells cannot be regenerated or replaced. Then the signs
of old age appear, like immune defects and arteriosclerosis. The scientist
Jerry Shaw found drastically shortened Telomeres with Progeria patients.
(SPIEGEL dated 22 January 1996, Title: "Faustischer Pact").
However, it cannot be said yet with certainty that the Telomere length
is the key to understanding and treating Progeria, or that it is the cause
of the aging process, since growing old is an incredibly complex process.
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