Scientists have discovered that they can
dramatically increase the life span of mice with premature aging and heart
disease by reducing levels of a protein called SUN1.
Scientists
have discovered that they can dramatically increase the life span of mice with
premature aging disease and heart disease by reducing levels of a protein
called SUN1.
This
research, published recently in the journal Cell, was a collaboration among the
A*STAR Institute of Medical Biology (IMB) in Singapore, the National Institute
of Allergy and Infectious Diseases (NIAID) in the United States, and the
Institute of Cellular and System Medicine in Taiwan.
Children
with progeria suffer symptoms of premature aging and mostly die in their early
teens from either heart attack or stroke. Individuals with a specific type of
heart disease, Emery-Dreifuss muscular dystrophy (AD-EDMD), suffer from muscle
wasting and cardiomyopathy, which eventually leads to heart failure.
Both
diseases are caused by mutations in Lamin A, a protein in the membrane
surrounding a cell’s nucleus which provides mechanical support to the nucleus.
SUN1 is a protein also found in the inner nuclear membrane, but there have been
no previous studies to show how SUN1 interacts with the Lamin proteins.
To
investigate if SUN1 had any involvement in diseases caused by mutations in
Lamin A, the scientists inactivated SUN1 in mouse models developed for progeria
and AD-EDMD. Instead of the short life spans commonly observed in these mouse
models, the mice lived twice (progeria) and thrice (AD-EDMD) as long compared
to when SUN1 levels were high.
“We
actually expected that knocking out Sun1 in these mouse models would worsen
their conditions and cause them to die faster but surprisingly we observed the
opposite,” said IMB graduate student and co-author Rafidah Abdul Mutalif.
As
mutations in Lamin A are frequently reported in hereditary cardiomyopathies,
the scientists hope that their research findings may lead to a potential
therapy for other forms of heart disease.
The
article can be found at: Chen CY et al. (2012) Accumulation of the Inner
Nuclear Envelope Protein Sun1 Is Pathogenic in Progeric and Dystrophic
Laminopathies.
Source: A*STAR.
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