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Zheng Xiao-Dong
HEMOPHILIA. A
CURIOUS
DISEASE
OF
THE ROYAL
FAMILIES
A small defect in a single human
gene
(the
basic DNA unit
for
inherit-
ance), and the resulting absence of
a
key blood-clotting protein it encodes,
can
make
to a person
bleed
to
death.
The disease
is
called
hemophilia. The
distinctive inheritance pattem
of
the dis-
ease, generally only males are afflicted
but
females may
be
carriers,
was
first
described early
in
19th
century.
The
most
celebrated carrier was Queen Vic-
toria.
One of her sons was hemophilic
and at least two of her daughters were
carriers.
Through
the
marriages of her
daughters'
Queen
Victoria's
mutant
gene spread to
the
royal
families
of
Ger-
many, Russia and
Spain.
lf
a
severely
ill
hemophiliac is not treated, he may
suffer internal
hemorrhaging
after
a
minor bump. He will probably die at an
early age from the effects of
a
bleeding
crisis.
Fortunately hemophiliacs
can
presently
be
treated with the missing
protein, which
is
made
by
genetic engi-
neering.
When the bioengineered pro-
tein
becomes commercially available
and inexpensive,
hemophiliacs
will
be
free from the menace of blood inlusion
and transfused infection.
Those
hemophiliacs who live in underdevel-
oped countries,
will
receive effective
treatment
lor
the first time.
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