Hunter James Kelly Research Institute will open on the University at Buffalo’s downtown medical campus. It honors Jim, Jill and their late son Hunter James Kelly Jr. who died from Krabbe Disease at age eight. Lawrence Wrabetz and Laura Feltri are two neuroscientists with expertise in myelin (the white sheath that protects brain nerve fibers).
Research
Researchers were recently granted a $2 Million grant to advance research of Krabbe disease, an extremely rare neurological disorder affecting newborns that often proves fatal. The funding will go to the Hunter James Kelly Research Institute at University at Buffalo recently established by Jim and Jill Kelly to honor their son Hunter James Kelly; it forms part of their New York State Center of Excellence for Bioinformatics and Life Sciences on Buffalo Niagara Medical Campus.
The funding will support a five-year research initiative focused on understanding the genetic basis for Krabbe disease and developing effective therapies to treat it. Ultimately, the goal is to find a cure for Krabbe, which damages myelin sheaths that protect brain nerve fibers and facilitate communication between them.
HJKRI has recruited two internationally-respected neuroscientists from Italy to assist in its research. Lawrence Wrabetz, head of myelin biology unit at San Raffaele Scientific Institute in Milan; Laura Feltri, leader of neuroglia research group; both have agreed to relocate their labs from Italy to work alongside scientists at HJKRI.
Both scientists are accomplished researchers of myelin. Not only can their expertise benefit those battling Krabbe disease or related leukodystrophies, but it will also benefit patients suffering from multiple sclerosis, stroke and other conditions characterized by white matter destruction.
HJKRI will be launched with an initial pledge of up to $3 Million from the Kelly Foundation and matched by UB, and used to establish its technology infrastructure within their Center of Excellence for Bioinformatics and Life Science – where various research initiatives will take place at HJKRI – expected to begin sometime this fall. A consortium of experts from around the country and world will oversee HJKRI.
Education
The institute has created a scholarship fund to assist medical and scientific students who wish to pursue degrees. Anyone seeking more information on applying can reach out directly to the institute for more details.
Jim and Jill Kelly’s son Hunter was diagnosed with Krabbe leukodystrophy (Krabbe disease), a rare genetic disorder of the nervous system which often proves fatal within months of birth. Despite all efforts made by Jim and Jill Kelly to save him, Hunter passed away at 8 years old in 2005 despite their best efforts at saving him. To honor Hunter James Kelly Research Institute – established in his memory to advance research for finding a cure – in memory of Hunter’s memory and to advance research for research of Krabbe disease research.
Researchers from the institute are focused on understanding myelin, which protects nerve fibers in both the brain and spinal cord, while simultaneously exploring genetic causes of neurodegenerative conditions like Krabbe disease and others.
Lawrence Wrabetz, MD, the Director of Myelin Biology Unit at San Raffaele Scientific Institute in Milan Italy and Laura Feltri MD who heads Neuroglia Division have both joined HJKRI as Directors. Both neuroscientists possess significant background and expertise in basic and translational myelin research and will work as part of a team at this new New York State Center of Excellence for Bioinformatics and Life Sciences located on Buffalo Niagara Medical Campus.
Outreach
The Kelly family established their foundation in memory of Hunter Kelly, who died at 8 from Krabbe disease, an incurable genetic disorder affecting his nervous system. Since 2005, their efforts have focused on raising awareness for Krabbe disease and leukodystrophies, as well as encouraging research for universal newborn screening.
Newborn screening involves performing a simple non-invasive blood test on infants at birth to identify potentially life-threatening disorders which can be detected using an analysis of a sample from their heel. As one of the largest public health programs nationwide, nearly 4.2 million tests are completed annually through newborn screening programs like these.
This research institute, funded jointly by the Kellys and UB, will be housed within their New York State Center of Excellence for Bioinformatics and Life Sciences on the Buffalo Niagara Medical Campus. Here, researchers will research remyelination techniques as well as biology and pathophysiology of Krabbe disease and related leukodystrophies with an aim of correcting gene mutations that cause such conditions, stopping its progressing, as well as developing effective treatments.
Events
At the Genetics, Genomics and Bioinformatics graduate program’s seventh annual Research Day event, several students and labs participated. Four students gave oral presentations while 10 displayed research posters – with prizes awarded to those displaying excellent work in each category.
Roosevelt House hosted this event, honoring Franklin and Eleanor Roosevelt’s legacy with its building’s transformation from Hunter College into a public policy institute in 2010. Roosevelt House serves three main purposes: teaching students in public policy and human rights studies, supporting faculty research initiatives, and encouraging creative dialogue.
HJKRI researchers recently received millions of dollars in federal funding to advance research on Krabbe disease, an inherited leukodystrophy and hereditary myelin sheath disorders such as Charcot-Marie-Tooth (CMT) disease. Congressman Brian Higgins from this area announced this funding.
Studies of hereditary neurological disorders have made strides over recent years, with major advancements made in natural history studies of various forms of these conditions, molecular pathways involved with them, improvements to newborn screening protocols for these conditions and possible therapies including myelin repair for neurodegenerative conditions.
At this year’s Hunter James Kelly Research Institute Family and Medical Symposium, world-class researchers working on Krabbe disease and other hereditary neurological diseases will come together to present their work to those affected by it. It’s free for families thanks to a grant from Ralph C Wilson Foundation; Hunter’s Hope’s Helping Hand Grant Travel Assistance program covers travel costs for attendees.
