---
title: "Hunter James Kelly Research Institute"
url: https://alsuprun.com/blog/kelly-research-technologies/hunter-james-kelly-research-institute-5/
author: "Editorial"
date: 2026-09-20T08:31:55+00:00
categories: ["kelly research technologies"]
tags: []
---

# Hunter James Kelly Research Institute

Research at the institute named after the son of former Buffalo Bills quarterback Jim Kelly and his wife, Jill, will focus on understanding myelin — the fatty insulation that enables nerve cells to communicate with each other. The work could improve treatments for diseases that destroy myelin, such as Krabbe disease and Charcot-Marie-Tooth disease.

 

## About HJKRI

 Research at the [Hunter James Kelly Research Institute](https://alsuprun.com/) (HJKRI) is focused on remyelination techniques and the biology and pathophysiology of Krabbe Disease and other leukodystrophies, as well as other diseases involving white matter destruction such as multiple sclerosis, stroke and Charcot-Marie-Tooth neuropathies. This is expected to lead to strategies for the correction of the genetic defect in these conditions, as well as the development of effective therapies.

 The HJKRI was established in 1997 by Buffalo Bills Hall of Fame quarterback Jim Kelly and his wife, Jill, after their infant son, Hunter, was diagnosed with the inherited, fatal disease Krabbe leukodystrophy, also known as globoid cell leukodystrophy. It is one of only a few research centers in the world with an exclusive focus on myelin — the sheath that protects brain nerve fibers and allows them to carry messages quickly and accurately.

 [![Rejuvenate your whole body & balance your health without medications - now remotely!](https://alsuprun.com/blog/wp-content/uploads/BioresonanceTopAd.png)](https://www.bioresonance.rent) A study published this week in the Proceedings of the National Academy of Sciences by HJKRI scientists sheds new light on the causes of diabetic neuropathy, a painful condition that affects millions of people in the United States and elsewhere and can lead to limb amputation if left unchecked. The UB researchers report that studying the molecular processes that cause some forms of neuropathy could accelerate the discovery of therapies.

 Laura Feltri and Lawrence Wrabetz, professors of biochemistry and neuroscience at the Jacobs School of Medicine and Biomedical Sciences at UB, are co-directors of the HJKRI. Their laboratory at the San Raffaele Scientific Institute in Milan, Italy, has received major funding from the Kelly Foundation and other sources. Feltri and Wrabetz have recently received additional NIH funding to explore the use of peripheral nervous system tissue as a window into Krabbe disease.

 

## Krabbe Disease

 Krabbe disease, which is part of a group of genetic disorders called leukodystrophies, causes a breakdown in the myelin sheath that helps messages travel quickly between nerve cells in the brain and spinal cord. It is usually fatal before a child turns five. Jim and Jill Kelly’s son, Hunter, battled the inherited condition that’s also known as Globoid Cell Leukodystrophy. Hunter’s Hope was established to raise awareness and fund research to find cures for this devastating disease.

 [![](https://alsuprun.com/blog/wp-content/uploads/ForeverYoungYouWannaBe.png)](https://alsuprun.com/services.html#Contact) Early symptoms of Krabbe disease include irritability and difficulty eating in infants. In some cases, children with the disease experience seizures, vomiting, a slowing of mental development and deafness or blindness.

 Because the disorder is so rare, it can be difficult to diagnose. Newborn screening is critical but few states currently test for this genetic condition. MRI scans that show the loss of protective myelin, as well as comprehensive neurological exams based on the patient’s reported symptoms, can help with diagnosis.

 A treatment that has shown promise is hematopoietic stem cell transplant (HSCT), which involves replacing unhealthy, defective blood cells with healthy ones. Hematopoietic stem cells can become all types of blood cells, including those that populate the brain and help make myelin. HSCT is most effective when given before the onset of symptoms.

 HJKRI researchers are using remyelination techniques to study myelin, which could lead to therapies that improve the quality of life for people living with Krabbe disease and other leukodystrophies. The work may help patients with multiple sclerosis and other diseases involving white-matter damage as well.

 Emotional support is critical for families dealing with a Krabbe disease diagnosis. Family members need a space to express fears, frustrations and hopes with others who understand the complexity of this devastating disorder. Counseling services and support groups can help. In addition, establishing long-term care plans can eliminate stress when significant decisions need to be made as the disease progresses. This includes identifying a facility that can provide specialized care and is equipped to handle complex conditions like leukodystrophies. In the future, gene therapy may offer even more hope for those suffering from this disease.

 

## Research Collaborations

 [Hunter James Kelly Research Institute](https://alsuprun.com/), or HJKRI, is home to scientists conducting research on myelin, a fatty insulator that enables communication between nerve cells in the central and peripheral nervous systems. Scientists also study how damage to myelin occurs and how it may be repaired. The HJKRI is part of UB’s New York State Center of Excellence in Bioinformatics and Life Sciences on the Buffalo Niagara Medical Campus in downtown Buffalo. The research is funded by a generous grant from the National Institutes of Health.

 [![](https://alsuprun.com/blog/wp-content/uploads/RadionicMerch.png)](https://alsuprun.com/merch.html) Congressman Brian Higgins recently announced federal funding to enhance newborn screening for Krabbe disease, an inherited fatal disorder of the central and peripheral nervous system caused by lack of an enzyme needed to make myelin. The work will be carried out by a team at UB, including researchers in the department of biostatistics and the department of neurology in the Jacobs School of Medicine and Biomedical Sciences.

 Lawrence Wrabetz, MD, head of the myelin biology unit at San Raffaele Scientific Institute in Milan, Italy, and Laura Feltri, who heads the neuroglia unit at that institution and is Wrabetz’s wife, have been recruited to UB’s HJKRI to work with Feltri and other UB scientists on Krabbe disease research.

 

## Funding Opportunities

 Students and laboratories in the Genetics, Genomics and Bioinformatics graduate program (GGB) shared their research during the seventh annual GGB Research Day. Four PhD students delivered oral presentations and 10 presented research posters during the event, held in the HJKRI auditorium. Cash prizes of $100 each were awarded to the best presentations.

 UB researchers are among only a handful of groups in the world that specialize in myelin research, a field focused on diseases like Krabbe disease and Charcot-Marie-Tooth (CMT) disease that result from misfolded proteins. Congressman Brian Higgins, D-New York 26, has earmarked funding to help researchers continue to make headway in understanding how myelin forms and the role it plays in diseases like Krabbe and CMT.

 New research reveals for the first time that Schwann cells — which surround axons within nerves like a glove covers a hand — can come to the rescue of axons damaged by myelin destruction. The work was led by M. Laura Feltri, MD, and Lawrence Wrabetz, MD, co-directors of the [Hunter James Kelly Research Institute](https://alsuprun.com/) and professors of neurology and biochemistry in the Jacobs School; and Daesung Shin, PhD, a research assistant professor at the HJKRI.

 The HJKRI is recruiting a postdoctoral research fellow to join its team and study the signals that control the differentiation and myelination of oligodendrocytes in normal and pathological conditions such as Multiple Sclerosis. This is an excellent opportunity to pursue independent and creative research in a collaborative environment.

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