NEWS RELEASE
January 25, 1996
 
Contact: TEL (619) 455-6480
       :
       : Terry Gach, x3204
       : tgach@ljcrf.edu
       :
       : Nancy Beddingfield, x3209
       : nbedding@ljcrf.edu
 
 
BREAKTHROUGH IN LOU GEHRIG'S DISEASE
Embargoed for release 5:00 PM Eastern Time On January 25, 1996
 
LA JOLLA, CA. -- Results from the laboratories of Dr. Dale E. Bredesen at
La Jolla Cancer Research Foundation and Dr. Joan Valentine at UCLA reported
in the January 26th issue of Science offer the first understanding of the
initiation of an inherited form of amyotrophic lateral sclerosis (ALS),
also known as Lou Gehrig's Disease.  These results further suggest the
possibility that existing, FDA-approved drugs, known as copper chelators,
might prove useful as preventive therapy for this form of ALS.
 
ALS is an age-dependent, neurodegenerative disorder which typically
afflicts individuals in middle adult life, leading to paralysis and death
within 3-5 years.   One person in 10,000 is afflicted with ALS.  Ten-15% 
of these cases are inherited and of these inherited cases, 20-25% are
associated with mutations in SOD1, the gene that encodes human copper-zinc
superoxide dismutase (SOD).
 
Normally, SOD destroys toxic molecules called free radicals, keeping cells
healthy.  Surprisingly, despite the mutations many patients with Lou
Gehrig's Disease were found to have normal SOD activity, leading to a
mystery:  why do these patients develop ALS?
 
Drs. Bredesen and Valentine discovered that, whereas the SOD activity was
frequently left untouched by the mutations, a new property was conferred on
the SOD by these mutations, leading to the rapid aging and degeneration of
the cells by oxidation (which is similar to rusting).  Most importantly,
this effect could be blocked by two different drugs, offering new hope for
treatment.
 
"I believe this is another important step in helping us to understand the
pathogenesis of this terrible disease," said Robert Abendroth, chairman of
the Research Committee of the ALS Association based in Woodland Hills,
California.  "Dr. Bredesen provides a hypothesis to the most frequently
asked question by ALS investigators since the indication of the SOD1 link
with familial ALS -- how does the mutant enzyme lead to ALS?  Dr.
Bredesen's answer is particularly significant because of its long range
therapeutic implications.  The ALS Association is especially pleased that
two of its research grant recipients, Dr. Bredesen and the UCLA team of
Drs. Joan Valentine and Edith Gralla, have collaborated on this important
study."
 
Dale E. Bredesen, M.D., is the Leader of the Program on Aging at the La
Jolla Cancer Research Foundation.  The La Jolla Cancer Research Foundation
is an independent, non-profit research institute involved in understanding
and controlling the biological processes of cancer and other diseases.  The
Foundation is one of 10 Basic Science Cancer Centers in the nation, so
designated by the National Cancer Institute.  More information is available
from the La Jolla Cancer Research Foundation's home page:
http://pines6.ljcrf.edu:6530/
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