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New mouse model will aid research on premature aging syndrome Eurekalert, May 14, 2003Researchers
from the National Cancer Institute (NCI) have developed a mouse model of
the premature aging syndrome known as Hutchinson-Gilford Progeria Syndrome
(HGPS), according to a report appearing in the journal Nature. Researchers
hope the mouse model will facilitate a better understanding of the fatal
syndrome, as well as provide clues to the normal aging process. Currently, there is
no treatment for progeria, and children with the rare condition usually
die of heart disease in their early teens. Although normal at birth,
children with progeria begin to develop growth retardation, thinning skin,
and fragile bones as young as 18 months. "The
similarities between mice with this particular mutation and patients with
progeria are remarkable," said Colin Stewart, Ph.D., of NCI's Center
for Cancer Research, the senior investigator on the study. "Now that
we've identified the critical gene and have an animal model that mimics
progeria, we have powerful tools for studying both the aging process and
this devastating disease." The results of the
animal study come less than a month after the announcement that scientists
have discovered the gene responsible for progeria in children. Studies
published in March in the journals Science and Nature described a single
inaccuracy in the Lamin A (Lmna) gene that appears to account for the
syndrome. The gene produces structural proteins known as lamins, which are
found in the cell nucleus. In the new study, NCI researchers report that
mice with a specific mutation in the same gene have symptoms remarkably
consistent with those of progeria patients. Although
indistinguishable from their littermates at birth, mice with the Lmna
mutation develop severe growth retardation early in life and die within
five weeks, whereas normal mice generally live up to two years. Like
progeria patients, mice in the study showed signs of premature aging. In the mice,
accelerated aging was most apparent in the skin, which thinned
dramatically and lost hair. Researchers also observed reduced growth or
degeneration of the heart and skeletal muscles in mutant mice. Similarly,
the mice had either incomplete development of the skeleton or a premature
loss of bone mass, also characteristic of children with progeria. Mice
with the Lmna mutation shared many other symptoms with progeria patients,
including a slight waddling gait, abnormal teeth, and incomplete sexual
maturation. The Lmna gene codes
for A- and C-type lamins, components of the fibrous network lining the
inside of the cell nucleus called the nuclear lamina. The mutant form of
the gene associated with progeria symptoms causes disruption in the
structure of the cell nucleus. Studying the relationship between progeria
symptoms and this abnormality could help researchers understand the
cellular processes associated with aging. Copyright
© 2002 Global Action on Aging
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