Lawrence Wrabetz and Laura Feltri, two highly esteemed neuroscientists, have joined the University at Buffalo Hunter James Kelly Research Institute as researchers in partnership with Hunter’s Hope Foundation – established by Buffalo Bills Hall-of-Famer Jim Kelly after their infant son Hunter Kelly died of Krabbe leukodystrophy – one of several diseases which destroy myelin sheaths that protect nerve cells – that affect his nerves.
Research
Hunter James Kelly Research Institute conducts research that is dedicated to disorders involving myelin, in order to develop more effective treatments. Myelin is an insulating sheath that encases nerve fibers throughout the brain and peripheral nervous system, enabling electrical signals to travel quickly through them. HJKRI was established to honor Hunter Kelly, former Buffalo Bills quarterback who died of Krabbe disease (also known as Globoid Cell Leukodystrophy). This inherited disorder is caused by lack of an enzyme needed to produce myelin; research conducted at HJKRI seeks to understand and find cures for Krabbe disease as well as similar hereditary myelin diseases such as Charcot-Marie-Tooth (CMT) disease.
Researchers from UB have discovered an important mechanism that may explain why myelin sheaths sometimes break down and lead to neuropathy – an illness which impacts millions of people and has even resulted in limb amputation if left untreated. A study published this week in Proceedings of the National Academy of Sciences by HJKRI investigators highlights this critical role played by an important protein complex called mTOR that regulates cell metabolism and growth.
Researchers in M. Laura Feltri’s lab at UB are studying where Schwann cells — which encase nerve axons like gloves do — connect to myelin sheaths. Their team has discovered that myelin sheaths may be more fragile than previously assumed and Schwann cells often come to their rescue when damaged axons need protecting.
UB faculty members have received over $2 Million in new National Institutes of Health funding for research into Krabbe disease and related hereditary neurological conditions that involve myelin breakdown. These grants form part of a $1.5 billion investment by both UB and the federal government that will help advance medical research in high-impact fields like cancer, cardiovascular disease and neuroscience that will stimulate economic development while improving patient care.
Education
Hunter James Kelly Research Institute was established to honor Jim Kelly, Hall-of-Famer Quarterback. Hunter died at eight from Krabbe Disease (formerly Globoid Cell Leukodystrophy), an inherited disorder which prevents the body from producing enough myelin to electrically connect nerve cells properly – myelin is white matter that protects brain nerve fibers from electrical impulses.
Lawrence Wrabetz of San Raffaele Scientific Institute in Milan, Italy and Laura Feltri from its neuroglia unit have been recruited as leaders for HJKRI and will begin transitioning their labs this fall.
Outreach
Congress gives newborn screening research an impressive boost with its establishment of the Hunter Kelly Newborn Screening Research Program. This park is named in memory of Jim Kelly and Jill’s son, Sean Kelly, who tragically passed away at eight years old from Krabbe disease, an incurable degenerative nervous system disorder. This program, conducted in collaboration between NICHD and Hunter’s Hope Foundation(link is external), seeks to identify additional conditions to include on newborn screening lists, develop research management strategies for those identified, sponsor education programs for providers, as well as develop techniques that may remyelinate white matter destruction such as multiple sclerosis and stroke. HJKRI scientists also are developing techniques that might remyelinate multiple sclerosis patients as well as those suffering stroke.
Funding
The University at Buffalo was recently awarded over $2 Million by The National Institutes of Health to support research into Krabbe disease, an infantile form of malignancy often fatal for newborns. Research could eventually lead to a cure for neurodegeneration, which destroys myelin-coated nerve fibers in the brain and damages their protective sheath known as myelin. Laura Feltri, MD, head of neuroglia unit at San Raffaele Scientific Institute in Milan, Italy; and Lawrence Wrabetz, PhD, director of Hunter James Kelly Research Institute established at UB with donations from Hall-of-Famer quarterback Jim Kelly and his wife Jill in 2004; will conduct the research at UB this fall and be joined by additional scientists recruited for inclusion at their institute.
