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From the inception of Hunter's Hope in 1997, an overarching goal of
the Foundation has been to fund research into the cause, prevention,
treatment and clinical care for children suffering from Krabbe and other
leukodystrophies.
To that end, Hunter's Hope entered into an
agreement in 2008 with the University at Buffalo
School of Medicine, to create the Hunter James Kelly Research Institute.
All Foundation funded research, including both clinical and basic science, is coordinated through the Institute, which
is located in the New York State Center of Excellence in Bioinformatics and Life Sciences (CoE), a prominent part of the Buffalo Niagara Medical Campus.
The mission of the HJKRI
is to study myelin and its diseases.
Myelin is the fatty insulation around axons (the wires of the nervous
system) in the brain, spinal cord and nerves. The HJKRI incorporates both basic and
clinical science programs.
Basic research is
based on a multi-disciplinary approach to understand how myelin is formed, how
it is damaged in disease, and how it may be repaired. In particular, HJKRI will study disease
mechanisms of Krabbe Disease in order to formulate therapies.
Clinical research
analyzes information revealed by newborn screening for Krabbe Disease and other
leukodystrophies. The World Wide Registry for patients with Krabbe Disease aims
to improve diagnosis and prognosis, and to provide outcome measures for
clinical trials.
This integrated
approach, together with a critical mass of resources and investigators, will
generate information that will promote effective treatment strategies not only
for children with leukodystrophies, but also patients with Multiple Sclerosis, stroke,
neuropathies and other diseases in which myelin is damaged.
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