More reviews online at
at 38min and 1 hour 36
http://www.bbc.co.uk/iplayer/b007qmxj/Saturday/console/
http://www.bbc.co.uk/blogs/bollywood/2009/12/movie_magic.html#more
Mr,Balakrishnan the director,was counselled by the Progeria society
of India,that the movie should not be about the disease Progeria or
premature ageing,because Progeria is a rare disease in India,and very
little is known medically about this disease and its an irreversible
condition,with no known cure,and they did not want the wrong message
to be sent to the public when so little info available,the movie
should only concentrate about the life of Auro or Aurobindo,where the
condition is relegated to the background.
It appears that a combination of alendronate and statin may stop
aging
http://www.youtube.com/watch?v=8D4S3pDjPiU
wikipedia
It was hypothesized that part of the reason that treatment with an
FFI such as alendronate is inefficient is due to prenylation by
geranylgeranyltransferase. Since statins inhibit
geranylgeranyltransferase, the combination of an FFI and statins was
tried, and markedly improved "the aging-like phenotypes of mice in the
metalloproteinase ZMPSTE24, including growth retardation, loss of
weight, lipodystrophy, hair loss, and bone defects".[29]
It is a genetic condition that occurs as a new mutation and is not
usually inherited, although there is a uniquely inheritable form. This
is in contrast to another rare but similar premature aging syndrome,
dyskeratosis congenita (DKC), which is inheritable and will often be
expressed multiple times in a family line.[4]
Classical Hutchinson-Gilford Progeria Syndrome is almost never
passed on from parent to child. It is usually caused by a new
(sporadic) mutation during the early division of the cells in the
child. It is usually genetically dominant; therefore, parents who are
healthy will normally not pass it on to their children.[6] Affected
children rarely live long enough to have children themselves.
There have been only two known cases in which it became evident that a
healthy parent can carry the LMNA mutation that causes progeria. A
family from India has five children with progeria
Lamin A
Nuclear lamin A is a protein scaffold on the inner edge of the nucleus
that helps organize nuclear processes such as RNA and DNA synthesis.
Prelamin A contains a CAAX box at the C-terminus of the protein (where
C is a cysteine and A is any aliphatic amino acids). This ensures that
the cysteine is farnesylated and allows prelamin A to bind membranes,
specifically the nuclear membrane. After prelamin A has been localized
to the cell nuclear membrane, the C-terminal amino acids, including
the farnesylated cysteine, are cleaved off by a specific protease. The
resulting protein is now lamin A, is no longer membrane-bound, and
carries out functions inside the nucleus.
In HGPS, the recognition site that the enzyme requires for cleavage of
prelamin A to lamin A is mutated. Lamin A cannot be produced, and
prelamin A builds up on the nuclear membrane, causing a characteristic
nuclear blebbing.[25] This results in the premature aging symptoms of
progeria, although the mechanism connecting the misshapen nucleus to
the symptoms is not known.
A study that compared HGPS patient cells with the skin cells from LMNA
young and elderly human subjects found similar defects in the HGPS and
elderly cells, including down-regulation of certain nuclear proteins,
increased DNA damage, and demethylation of histone, leading to reduced
heterochromatin.[26] Nematodes over their lifespan show progressive
lamin changes comparable to HGPS in all cells but neurons and
gametes.[27] These studies suggest that lamin A defects contribute to
normal aging.
The Indian Bollywood movie Paa, released in December 2009, has
its story line around progeria (through the life of a twelve year old
boy Auro).
...