Swedish Research: New findings may stop the early aging
Friday, 17 May 2013
Progeria otherwise know in as HGPS (Hutchinson-Gilford Progeria
Syndrome) an extremely rare, fatal genetic condition which affects
children and gives them an appearance of accelerated aging seems to be
opening up to the route of cure led by researchers in Sweden.
Researchers from Sahlgrenska Academy at the University of Gothenburg
believe in their findings that Pharmaceuticals that inhibit a specific
enzyme may be useful in treating progeria, or accelerated aging in
children. A new study performed at the Sahlgrenska Academy indicates
that the development of progeria in mice was inhibited upon reducing
the production of this enzyme.
Therefore the researchers have identified a mechanism that blocks an
enzyme associated with accelerated aging according to their finding
published today in the journal Science.
"This study is a breakthrough for our research group after years of
work," said senior author Martin Bergö in a news release. "When
we reduce the production of the enzyme in mice, the development of all
the clinical symptoms of progeria is reduced or blocked."
The disease is very rare and not hereditary. Only a few hundred
children worldwide are affected. Researchers in Gothenburg are now
cooperating with a university in Singapore that has developed two
possible medications, according to Swedish media reports.
More about Progeria and the research
Progeria is a rare genetic childhood disorder characterized by the
appearance of accelerated aging. The classical form of progeria, called
Hutchinson-Gilford Progeria Syndrome (HGPS), is caused by a spontaneous
mutation, which means that it is not inherited from the parents.
The progeria mutation occurs in the protein prelamin A and causes it
to accumulate in an inappropriate form in the membrane surrounding the
nucleus. The target enzyme, called ICMT, attaches a small chemical
group to one end of prelamin A. Blocking ICMT, therefore, prevents the
attachment of the chemical group to prelamin A and significantly
reduced the ability of the mutant protein to induce progeria.
"We are collaborating with a group in Singapore that has developed
candidate ICMT inhibitor drugs and we will now test them on mice with
progeria. Because the drugs have not yet been tested in humans, it will
be a few years before we know whether these drugs will be appropriate
for the treatment of progeria," Martin Bergö explains.
Although there are only a few hundred children in the world with
progeria, the disease, children, and research have attracted a great
deal of attention.
"The reason is obvious: the resemblance between progeria patients
and normally-aged individuals is striking and it is tempting to
speculate that progeria is a window into our normal aging process. The
children develop osteoporosis, myocardial infarction, stroke, and
muscle weakness. They display poor growth and lose their hair, but
interestingly, they do not develop dementia or cancer," says Martin
Bergö, who is also studying the impact of inhibiting ICMT on the normal
aging process in mice.
- Mohamed X. Ibrahim, Volkan I. Sayin, Murali K. Akula, Meng Liu,
Loren G. Fong, Stephen G. Young, and Martin O. Bergo.(2013) Targeting
Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of
Progeria. Science, 16 May 2013.
- Swedish Television
- Sahlgrenska Academy