|
Features: March 18, 2002
NIH Awards Colón $1 Million for
Research Into Lou Gehrig's Disease
 The
cause of Lou Gehrig's Disease (amyotrophic lateral sclerosis
or ALS) has remained elusive since it brought down one of
baseball's greatest players 60 years ago.
According to Wilfredo "Freddie"
Colón, ALS starts "when good proteins go bad."
Understanding just why they go bad is a necessary first
step toward developing medicines that will help ALS patients
live with a manageable disease instead of a death sentence.
| |
ALS starts "when good proteins
go bad." Understanding just why they go bad is
a necessary first step toward developing medicines that
will help ALS patients live with a manageable disease
instead of a death sentence.
—Wilfredo "Freddie"
Colón—
|
The Rensselaer biochemist's research recently
earned a $1 million, four-year grant from the National Institutes
of Health (NIH).
The grant will support Colón's study
of the hereditary version of the disease, called familial
ALS (FALS). He is attempting to understand why mutants of
the enzyme superoxide dismutase (SOD1) fail and misfunction
in FALS.
On average, FALS strikes people who are
around 47 years of age, says Colón. Most patients
die within two to five years. But some patients, whose proteins
exhibit a different kind of mutation, experience a very
slow progression and can survive for as long as 18 years,
he says.
The existence of SOD1 mutants associated
with this "milder" form of FALS makes it an intriguing
biophysical and biochemical marker. Unlocking the mystery
of the pathogenic causes for FALS could also play an important
role for better understanding other neurogenerative disorders,
such as Parkinson's and Alzheimer's.
According to the National
Institutes of Health (NIH), ALS is the most common neuromuscular
disease worldwide. In the U.S. there are 20,000 people with
the disease and 5,000 people are diagnosed each year.
|
