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September 14; Woburn, Massachusetts
The Spastic Paraplegia Foundation (SPF) announces that it has
awarded $333,561 in research grants to three outstanding
scientists to help find the cures for Primary Lateral
Sclerosis (PLS) and Hereditary Spastic Paraplegia (HSP). The
SPF’s
Scientific Advisory Board rated their proposals as
outstanding opportunities to advance research and help find
the cures for PLS & HSP.
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| Bruce Horazdovsky,
Ph.D. |
Peter W. Baas,
Ph.D. |
Stephan Zuchner, M.D. |
The grant recipients, and the titles of their proposals, are
as follows:
Bruce Horazdovsky, Ph.D.
Bruce Horazdovsky, Ph.D., Associate Dean of the Mayo
Clinic College of Medicine, and senior consultant to the
department of biochemistry and molecular biology and the
Mayo Clinic Cancer Center (Rochester, MN) was awarded
$58,000 for his project entitled “Development of a
cell culture system to analyze defects associated with
Primary Lateral Sclerosis”.
Dr. Horazdovsky has helped to uncover the function of the
Alsin protein. (The
Alsin protein -- when defective -- causes juvenile onset
PLS.) His work on other similar proteins has been published
in numerous scientific journals.
Peter W. Baas, Ph.D.
Peter W. Baas, Ph.D., a professor in the department of
neurobiology and anatomy, in the College of Medicine, at
Drexel University (Philadelphia, PA) was awarded $140,000
($70,000 a year for two years) for his project entitled "Mechanistic
Basis of SPG4-based Hereditary Spastic Paraplegia”.
Dr. Baas has devoted his twenty-one year career in molecular
neurobiology to the study of the role of and transport of
microtubules in the axons of neurons. He is one of the
world’s leading scientists in that field. He has made major
discoveries about the role of a microtubule-severing protein
called
Katanin. (Spastin, a protein that causes the most common
form of HSP when defective, is also a microtubule severing
protein.) He has published eighty-five articles in
scientific journals.
Stephan Zuchner, M.D.
Stephan Zuchner, M.D., the director of the Center for
Human Molecular Genetics at the Miami Institute for Human
Genetics in the Leonard M. Miller School of Medicine (Miami,
FL) was awarded $135,561 ($67,148 the first year, and
$68,413 the second year) for his project entitled “Molecular
and genetic analysis of the SPG31 gene REEP1”.
Dr. Zuchner was the lead author of the paper announcing the
discovery of the
REEP1 gene – the third most common HSP-related gene. He
has published 29 articles in major scientific journals.
HSP and PLS are closely related disorders caused by the
degeneration of "upper motor neurons".
Some 24,000 people across the U.S. and Canada are estimated to be
affected by HSP. PLS is thought to affect 1-2 thousand
individuals, although it is difficult to estimate since it can
be difficult to differentiate from HSP and ALS in its early
stages. Both
disorders cause progressive spasticity and weakness in the legs,
causing patients to use canes, crutches or eventually
wheelchairs. PLS also affects the arms and hands and generally causes
speech difficulties. Very rare forms of HSP can
cause mental retardation, deafness, diseases of the retina,
dementia, ataxia (lack of muscle control), and epilepsy.
The SPF is a national, volunteer-driven,
non-profit organization dedicated to finding the cures for upper motor neuron
disorders and providing information and support services to
people affected by them.
For more information, please contact SPF at
information@sp-foundation.org or
visit the website at
http://www.sp-foundation.org.
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