News & Events

Study: Axon Degeneration Due to Sick Oligodendrocytes

Elisabetta Babetto, PhD, and Bogdan K. Beirowski, MD, PhD, are leading a study seeking to find novel therapeutic approaches to specifically protect axons from degeneration.

April 16, 2020

Jacobs School of Medicine and Biomedical Sciences researchers are seeking to improve understanding of the glial maintenance and support of axons — the very long cellular projections of neurons relaying electrical and biochemical signals in nerves and white-matter tracts of the nervous system.

Bogdan K. Beirowski, MD, PhD, assistant professor of biochemistry, and Elisabetta Babetto, PhD, senior research scientist in biochemistry and research assistant professor of pharmacology and toxicology, are leading the study.

As investigators at the Hunter James Kelly Research Institute, they have been awarded a five-year, $1.9 million grant from the National Institute of Neurological Disorders and Stroke for their study titled “Elucidating the Trophic Support of Long Axons by Metabolic Signaling in Oligodendrocytes.”

Graduate Student, Narayan Dhimal, Joins the Feltri Laboratory

Narayan Dhimal pictured with Doctoral students, MD-PhD students and mentors at the PhD Program in Biomedical Sciences’ third annual white coat ceremony.

PPBS White Coat Ceremony Honors Student Advancement

July 1, 2019

The PhD Program in Biomedical Sciences (PPBS) conducted its third annual white coat ceremony to recognize 19 students from the Class of 2018-2019 — 14 doctoral students and five MD-PhD students — who completed their first year in the program and are moving on to their research laboratory match.


14 PPBS Students Match into Research Lab

The PPBS students were cloaked by the faculty members into whose research labs they matched.

Pursuing New Direction to Understand Krabbe Disease

M. Laura Feltri, MD, and Lawrence Wrabetz, MD

May 7, 2019

The Hunter James Kelly Research Institute (HJKRI) has received more than $2 million in National Institutes of Health (NIH) funding, enabling researchers to pursue a new approach to Krabbe disease.

Research Will Pinpoint Cells Needing Correction

Co-principal investigators on the grant — co-directors of the HJKRI M. Laura Feltri, MD, professor of biochemistry and neurology, and Lawrence Wrabetz, MD, professor of neurology and biochemistry  — say the new approach focuses on the peripheral nervous system as a possible “window” into the disease.

“What’s exciting about this grant is that we are leading research on Krabbe disease in a new direction,” Feltri says. “Our research will pinpoint which cells need correction, using methods such as gene therapy, in order to cure the disease."

Jacobs Faculty Members Earn SUNY Chancellor’s Awards

M. Laura Feltri, MD


May 6, 2019

Four Jacobs School of Medicine and Biomedical Sciences faculty members have been selected as recipients of 2019 SUNY Chancellor’s Awards for Excellence.

The Chancellor’s Awards acknowledge and provide systemwide recognition for consistently superior professional achievement, and they encourage the ongoing pursuit of excellence.

Groundbreaking Research on Myelin Disorders

M. Laura Feltri, MD, professor of biochemistry and neurology, was awarded the Chancellor’s Award for Excellence in Scholarship and Creative Activities, which recognizes the work of those who engage actively in scholarly and creative pursuits beyond their teaching responsibilities.

UB Scientists Studying Krabbe’s Disease Take a Novel Approach

Norma Nowak, NYS Center of Excellence in Bioinformatics and Life Sciences, Executive Director; HJKRI Co-director M. Laura Feltri and Rep. Brian Higgins 

March 28, 2019

The funding allows UB researchers at the institute to pursue a new approach to Krabbe’s Disease, also called globoid cell leukodystrophy, a rare, neurological disease that afflicts newborns and is fatal.

This new direction, Higgins noted, comes about as a result of the continued investment in the institute, one of a handful of research institutes in the world with an exclusive focus on myelin and diseases of myelin, such as multiple sclerosis, demyelinating neuropathies and leukodystrophies, and how they may be treated.

Congressman Higgins Announces Over $2M in Federal Funding to Support Krabbe Disease Research at the Hunter James Kelly Research Institute at UB

Norma Nowak, NYS Center of Excellence in Bioinformatics and Life Sciences, Executive Director; HJKRI Co-director M. Laura Feltri and Rep. Brian Higgins 

March 25, 2019

New Grant Supporting 5-Year Project Is the Latest In A Series of Awards Furthering Krabbe Disease Research in WNY

Congressman Brian Higgins (NY-26) announced millions of dollars in federal funding to support the research of Krabbe leukodystrophy, known as Krabbe Disease (KD). A grant from the National Institutes of Health (NIH) in the amount of $433,627 in the first year and over $2 million over a five-year period has been awarded to M. Laura Feltri, MD and Lawrence Wrabetz, MD, co-directors of the Hunter James Kelly Research Institute at the University at Buffalo (UB). This is just the latest in a series of awards furthering Krabbe Disease research earned by the Institute this year. 

MD-PhD Student Awarded Prestigious NIH Fellowship

Chelsey Reed, PhD Student, HJKRI, Wrabetz lab

October 25, 2018

Chelsey Reed, a student in the MD-PhD Program, has been awarded a prestigious fellowship from the National Institutes of Health (NIH).

The Ruth L. Kirschstein National Research Service Award Fellowship (F30) is intended to enhance research and clinical training of promising predoctoral students who are matriculated in a combined MD/PhD training program and plan to pursue careers as physician-scientists.

Studying Cellular Stress Response Pathways

Reed is an MD-PhD student in the neuroscience program and is working at the Hunter James Kelly Research Institute (HJKRI) in the laboratory of Lawrence Wrabetz, MD, professor of neurology and biochemistry, and inaugural director of the HJKRI.

Axonal Degeneration Focus of Multiple Studies at HJKRI

Elisabetta Babetto, PhD, left, and Bogdan K. Beirowski, MD, PhD

August 3, 2018

Researchers, Elisabetta Babetto, PhD and Bogdan K. Beirowski, MD, PhD at the Hunter James Kelly Research Institute (HJKRI) have been awarded a pair of grants for the investigation of mechanisms underlying axonal degeneration in certain neurological disorders.

‘Underlying Mechanisms Poorly Understood’

A grant funded by the Muscular Dystrophy Association (MDA) was awarded to Bogdan K. Beirowski, MD, PhD, assistant professor of biochemistry.

Elisabetta Babetto, PhD, senior research scientist in biochemistry and research assistant professor of pharmacology and toxicology in the HJKRI, was awarded a grant funded by GBS/CIDP Foundation International.

HJKRI Collaborator: Sim Research Exploring New Drug Therapies for Multiple Sclerosis

Fraser J. Sim, PhD

March 28, 2018

Research led by Fraser J. Sim, PhD, associate professor of pharmacology and toxicology, that seeks to spur development of cellular and molecular therapies for adult demyelinating disease, such as multiple sclerosis, has gained funding from the National Institutes of Health.

Investigating Strategies to Improve Myelin Repair

In multiple sclerosis, impaired oligodendrocyte differentiation limits remyelination and leads to axonal atrophy and neurodegeneration, according to Sim, principal investigator on the grant.

“Drugs which block muscarinic receptors (MR) have been shown by us and others to improve remyelination and myelin repair in rodents,” he says.

“Understanding the mechanisms by which these drugs act and the role of muscarinic acetylcholine (ACh) signaling in oligodendrocyte progenitor cells (OPCs) is of paramount importance to the successful clinical translation of this approach.”

Pablo Paez promoted to Associate Professor

Pablo M. Paez, PhD

Spring 2018

We want to congratulate Dr. Pablo Paez, HJKRI researcher since 2012, who has been promoted to Associate Professor in the Department of Pharmacology and Toxicology in the School of Medicine and Biomedical Sciences, University at Buffalo.  

Dr. Paez was approved for this promotion, effective 7/1/2018, after demonstrating his achievements through the rigorous process of evaluation and review.

The criteria met by Paez included:

  • must hold the appropriate terminal degree or have equivalent scholarly or professional attainments. 
  • should have achievements in teaching and research or creative activity extending well beyond those involved in the attainment of the doctoral degree or its equivalent. 
  • continuing appointment as an Associate Professor
  • demonstrated a continued high level of performance as a teacher and researcher or creator, 
  • commitment to high scholarly standards, and evidence of effective participation in university and community service. 
  • quality of the research or creative activity of such an appointee should be unambiguous and unequivocal. 
  • substantial evidence of peer review that has been carried out in a manner characteristic of and appropriate to the discipline.
  • must demonstrate solid professional achievement and the potential to meet requirements for the rank of full professor. 
  • has the credentials to achieve continuing appointment as an Associate Professor in his or her discipline at the leading public research universities.


M. Laura Feltri, MD & Lawrence Wrabetz, MD

Summer 2017

Lawrence Wrabetz, MD, and M. Laura Feltri, MD, are world leaders in myelin research who have dedicated themselves to laying the groundwork for prognostic, diagnostic and treatment strategies for neurological diseases caused by white matter destruction in the nervous system.

Married for 21 years and research collaborators since 1990, the couple met in a neurology lab, where they discovered an affinity for shared research goals-and each other. "We're a good fit in the most important ways, and we're fortunate that we share a deep respect for science and a commitment to making a difference in diseases where there has been very little hope," says Feltri.

Researchers Study Novel Proteins’ Role in Myelination

M. Laura Feltri, MD

August 1, 2017

M. Laura Feltri, MD, professor of biochemistry and neurology, is leading research to determine whether a new family of molecules prevents demyelination and nerve degeneration in patients with peripheral nerve diseases.

Research Builds on 2014 Finding

The novel molecules, called prohibitins, are required for nerves to form correctly and remain healthy. 

Feltri and fellow researchers identified the prohibitins in 2014 using a newly discovered way to study the interface where cells in the myelination process connect. “To identify them, we used a innovative cell chamber assay to isolate the proteins that are located in the cellular part of Schwann cells that are used to contact neurons,” she explains. 

How does neuropathy happen? New research reveals a pathway and a possible therapeutic option

Oligodendrocytes with cortical neurons.

Fluorescence microscopy image of in vitro model of myelin sheath (red) formation by Schwann cells around neuronal axons (green). Cell nuclei are blue. Credit: Keit Men Wong, UB.

May 12, 2017

UB researchers studying myelination are discovering how metabolic diseases like diabetes may cause neuropathy.

BUFFALO, N.Y. — Diabetic neuropathy is one of the most common complications of diabetes. While not life-threatening, it affects millions in the U.S. and elsewhere, and leads to limb amputations if left unchecked. But the reasons why metabolic disease can lead to neuropathy, which is damage to the peripheral nervous system, have never been well-understood.

Now, in a paper published this week online in Proceedings of the National Academy of Sciences, researchers at the Hunter James Kelly Research Institute (HJKRI) at the University at Buffalo report on research that illuminates what causes some kinds of neuropathy and may reveal potentially powerful therapies.

PhD Student Development Program Gains Renewed Funding from NIH

Mohamed Sharif, a doctoral candidate in the HJKRI, Park Laboratory.

May 10, 2017

National Institutes of Health (NIH) training grant aimed at providing professional development resources and mentoring for doctoral students in several UB schools has been renewed for another five years.

The Initiative for Maximizing Student Development (IMSD) will provide $2.3 million in funding to train 20 new biomedical and behavioral scientists from underrepresented groups between now and 2021.

The institutional grant benefits doctoral students in the following schools and institutes: Jacobs School of Medicine and Biomedical Sciences, School of Dental Medicine, School of Pharmacy and Pharmaceutical Sciences, School of Public Health and Health Professions, College of Arts and Sciences, School of Engineering and Applied Sciences and Roswell Park Cancer Institute Graduate Division.

[see sections entitled: "Flexibility Valuable Benefit of Program" & "Study Groups Encourage Interaction Between Students"...which quotes HJKRI Park Laboratory PhD Student, Mohamed Sharif]

Pathway Identified in Myelinating Glia Offers Hope for New Therapies

(left to right) Doctoral student Keit Men Wong, Bogdan Beirowski, and Elisabetta Babetto.

Doctoral student Keit Men Wong (left), Bogdan Beirowski, MD, PhD, and Elisabetta Babetto, PhD, are studying a pathway that plays a key role in regeneration of myelin sheaths.

May 9, 2017

Researchers at the Hunter James Kelly Research Institute (HJKRI) are highlighting the critical role of a metabolic checkpoint kinase pathway in Schwann cells for the formation of myelin sheaths.

“We are encouraged by our findings and think that our discoveries could be exploited to regenerate myelin sheaths and nerve structure to help patients with neurological disorders.”

This pathway, centered around the molecule “mammalian target of rapamycin (mTOR),” is known to play a paramount role in the regulation of cell metabolism, growth and cell division, as well as aging.

Nerve cells talk to each other by transmitting electrical signals along communication cables called axons, which are enwrapped by insulating myelin sheaths. The myelin sheaths produced by Schwann cells keep axons energized and healthy, and they facilitate the propagation of electrical signals by a process known as saltatory conduction.

Graduate Students Compete in First 3 Minute Thesis Event

Medical school graduate students, from left, Nadav Weinstock, Danielle Twum, Lucie Kafkova and Chong Zhang participated in UB’s first 3 Minute Thesis competition.

April 20, 2017

Four graduate students from the Jacobs School of Medicine and Biomedical Sciences were among the 15 finalists of UB’s inaugural 3 Minute Thesis (3MT) competition.

African Trypanosomes, Krabbe Disease Among Topics

Nadav Weinstock, a fifth-year MD/PhD student, aspires to become a physician-scientist working on developing therapies for rare diseases that affect babies.

During his first two years of medical school, Weinstock became interested in global health and spent time providing medical care at local free health clinics and to underserved remote populations in the Himalayan Mountains of India.

In 2016, he was awarded an F30 fellowship from the NIH for his research on Krabbe Disease.

His adviser in the Medical Scientist Training Program is M. Laura Feltri, MD, professor of biochemistry and neurology.

Weinstock’s presentation was titled “Understanding Krabbe Disease.”

Santiago-Gonzalez awarded an ASN Young Investigator Educational Enhancement Travel Award

Lawrence Wrabetz, MD (left); Pablo M. Paez, PhD, and Diara Santiago-Gonzalez

March 19, 2017

Diara Santiago-Gonzalez, HJKRI graduate student in the Paez laboratory, was selected to receive a Young Investigator Educational Enhancement (YIEE) travel award to attend the American Society for Neurochemistry (ASN) meeting in Little Rock, Arkansas from March 18 to 22, 2017.

The YIEE award winners were recognized Sunday morning, March 19th before the sessions, at 8 am., according to Donna Osterhout, PhD, the Chair of the YIEE Selection Committee or the American Society for Neurochemistry.

Calcium Channel Research May Help Improve MS Therapies

Pablo M. Paez, PhD

December 14, 2016

More researchers have identified a critical step in myelination after birth that has significance for treating neurodegenerative diseases like multiple sclerosis (MS).

In MS and similar diseases, myelin — the protective coating that neurons need to function — becomes lost or damaged. 

New clues to myelination could help identify ways to intervene in neurodegenerative diseases

cell culture of oligodendrocytes interacting with neurons during myelination process.

Cell culture containing oligodendrocytes with cortical neurons. Immature oligodendrocytes (bright yellow) interact with neurons (orange) during the first steps of the myelination process. (Credit: Pablo Paez)

December 7, 2016

UB researchers have identified a critical step in myelination after birth that has significance for treating neurodegenerative diseases like multiple sclerosis, in which myelin is lost or damaged. Myelin is the protective coating that neurons need to function.

The preclinical research, published online in October in the Journal of Neuroscience, concerns oligodendrocytes, the cells that make myelin, and the progenitor cells that are their precursors.

The work involved the study of voltage-operated calcium channels, which are responsible for initiating many physiological functions.

How myelin-making cells mature

“Our findings show that these calcium channels modulate the maturation of oligodendrocytes in the brain after birth,” said Pablo M. Paez, PhD, an assistant professor in the Department of Pharmacology and Toxicology in the Jacobs School of Medicine and Biomedical Sciences at UB and a research scientist with the Hunter James Kelly Research Institute (HJKRI) at UB, where most of the work was done.

“That’s important because it’s possible that the activity of this calcium channel can be manipulated pharmacologically to encourage oligodendrocyte maturation and remyelination after demyelinating episodes in the brain,” he said.

Three MD/PhD Students Receive NIH F30 Individual Fellowships

Nadav Weinstock.

Nadav Weinstock

November 14, 2016

Three students in the Medical Scientist Training Program (MD/PhD) have received prestigious fellowships from the National Institutes of Health (NIH).

The Ruth L. Kirschstein National Research Service Award Fellowship (F30) is intended to enhance research and clinical training of promising predoctoral students who are matriculated in a combined MD/PhD training program and plan to pursue careers as physician-scientists.

“The three F30 fellowships represent a significant achievement for the students as well as for our MD/PhD training,” says Suzanne G. Laychock, PhD, director of the Medical Scientist Training Program and senior associate dean for faculty affairs and facilities.

The awards:

Research Focused on Krabbe Leukodystrophy

Title: “Cell Specific Ablation of Galc and the Pathogenesis of Krabbe Disease”
Principal investigator: Nadav Weinstock
Length of project: Four years
Total funding: $153,772

Weinstock is a fifth-year student who works in the lab of M. Laura Feltri, MD, professor of biochemistry and neurology.

Through his work at the Hunter James Kelly Research Institute (HJKRI), Weinstock developed a shared project on Krabbe Leukodystrophy (KL) with Lawrence Wrabetz, MD, HJKRI director and professor of neurology and biochemistry; and Daesung Shin, PhD, research assistant professor at HJKRI.

Weinstock aspires to become a physician-scientist in the field of pediatric neurology. His goal is to care for patients as a clinician while also conducting basic science research.


UB honors exceptional scholars, innovative teachers

M. Laura Feltri, MD.

M. Laura Feltri, MD

July 7, 2016

Fifteen of UB’s best and brightest teachers and researchers have been named recipients of the university’s 2016 Exceptional Scholar and Teaching Innovation awards.

All will be honored at the annual Celebration of Faculty/Staff Excellence, to be held on Oct. 20.

Research Shows Role of Mechanical Signaling in Myelination


M. Laura Feltri, MD

July 6, 2016

UB researchers led by M. Laura Feltri, MD, professor of biochemistry and neurology, have discovered that mechanical forces play a critical role in the formation of myelin. Their findings, published online June 6 in Nature Neuroscience, may signal a path to new therapies for multiple sclerosis and other myelin-related diseases.

Higgins announces $239,250 federal grant to UB for work at Hunter James Kelly Research Institute

Thomas Langan, MD.

Thomas Langan, MD

June 10, 2016

Grant aimed at early, accurate diagnosis of Krabbe disease, and aiding in the treatment of newborns.

Congressman Brian Higgins announced the State University of New York at Buffalo was awarded a federal grant totaling $239,250 from the U.S. Department of Health and Human Services toward the goal of enhancing the accuracy of newborn screening for Krabbe disease.

Myelin cells feel the force, UB researchers find

Myelination image of Yap and Taz proteins.

When two proteins required for myelination, Yap and Taz (green) are subjected to low mechanical stimulation, they stay in the cell cytoplasm (left) and don’t enter the nucleus (blue). But when subjected to higher mechanical stimulation,  both proteins enter the nucleus, turning it blue-green, (right), triggering myelination. (Credit: Y. Poitelon, Feltri lab)

June 6, 2016

Mechanical forces play a critical role in myelination, the formation of the protective coating that neurons need to function, researchers at the University at Buffalo have discovered.

The UB researchers found that Schwann cells, the cells that form myelin in the nervous system, respond to mechanical stimuli by activating certain molecules that are then transferred to the nucleus to trigger myelination.

The finding opens new possibilities in developing treatments for myelin-related diseases.

Physician Scientists Association Grants Local Chapter to UB

(from left to right) Chelsey Reed, Nadav Weinstock, and Lauren Burkard-Mandel.

Chelsey Reed, medical school liaison (left); Nadav Weinstock, treasurer; and Lauren Burkard-Mandel, president; are among the student leaders of UB’s local APSA chapter.

March 11, 2016

The University at Buffalo’s Medical Scientist Training Program (MD/PhD) has been named a local chapter of the American Physician Scientists Association (APSA).

APSA is a national organization dedicated to addressing the needs of future physician-scientists with respect to their training and career development

Park Receives $1.8M Grant to Study Transcription Factor in Myelination

Yungki Park, PhD.

With funding from the National Institute of Neurological Disorders and Stroke, Yungki Park is researching the fundamental mechanism of myelin regulatory factor.

October 20, 2015

Yungki Park, PhD, assistant professor of biochemistry, has received a five-year, $1.8 million grant from the National Institute of Neurological Disorders and Stroke to study a transcription factor key to developing and maintaining myelin in the central nervous system.


New Research Technique Boosts Understanding of Myelin Diseases

Yannick Poitelon, PhD (left), and M. Laura Feltri, MD.

Research by Yannick Poitelon, PhD (left), and M. Laura Feltri, MD, boosts understanding of myelin diseases such as multiple sclerosis.

October 6, 2015

Researchers at the Hunter James Kelly Research Institute (HJKRI) have discovered a new way to study the interface where cells in the myelination process connect — a method that may lead to a better understanding of myelin diseases.


New candidate drug 'dials down' protein synthesis to treat demyelinating diseases

Lawrence Wrabetz MD.

Lawrence Wrabetz, MD

April 21, 2015

UB researcher contributes to study on new candidate drug that may help treat misfolded protein diseases like CMT, Alzheimer’s and Parkinson’s.

“Motor function in the animals returned to normal, the amount of myelin destruction was reduced by 70 percent and myelin thickness improved remarkably” ...

Biochemist IDs Pathway Central to Stability of Nerve Axons

Bogdan K. Beirowski, MD, PhD.

Bogdan K. Beirowski, MD, PhD

December 15, 2014

A University at Buffalo biochemist led the first study to identify the liver kinase B1 (LKB1) pathway as a possible therapeutic target for neuropathies, including diabetic neuropathy.

(from left to right) Steven J. Fliesler, PhD, and Lawrence Wrabetz, MD.

Steven J. Fliesler, PhD (left) and Lawrence Wrabetz, MD

Vision, Neurology Experts Named UB Distinguished Professors

July 15, 2014  

Steven J. Fliesler, PhD, Meyer H. Riwchun Endowed Chair Professor of ophthalmology and professor of biochemistry, and Lawrence Wrabetz, MD, professor of neurology and biochemistry, have been named University at Buffalo Distinguished Professors, effective Sept. 1.

They are among five UB professors receiving this honor in 2014 in recognition of their scholarly distinction and leadership.

Yungki Park, PhD.

Yungki Park, PhD

5 Departments Welcome New Faculty

February 21, 2014

Department of Biochemistry--Yungki Park, PhD, is an assistant professor. Park's research aims to increase knowledge about how oligodendrocyte differentiation is regulated for central nervous system myelination. His work could provide a firm basis for developing more effective therapeutics for demyelinating diseases.

Lawrence Wrabetz giving state address .

Lawrence Wrabetz, MD, director of UB’s Hunter James Kelly Research Institute

UB Chosen to Co-Lead Major State Effort to Spur Genome Research

January 9, 2014  

Gov. Andrew M. Cuomo has announced that the University at Buffalo will co-lead a statewide effort to position New York State as a national leader in genomic medicine.

At his State of the State address, Cuomo said UB will partner with the New York Genome Center (NYGC) in Manhattan to accelerate recent advances in genomic medicine directly into clinical care.

As part of this effort, UB will receive $50 million to increase research capacities.

UB will provide NYGC - a consortium of 16 educational and research organizations - with expertise and supercomputing power.

UB Study Seeks Molecular Mechanism Leading to Myelination

Pablo Paez, PhD.

Pablo M. Paez, PhD

August 14, 2013

Pablo M. Paez, PhD, assistant professor of pharmacology and toxicology, will use a $1.74 million grant to study how cellular processes involving calcium channels contribute to myelination and myelin pathology.

Myelination Researcher Honored as Distinguished Postdoc Mentor

M. Laura Feltri, MD.

M. Laura Feltri, MD

July 30, 2013  

M. Laura Feltri, MD, professor of biochemistry, has been honored with the 2013 Distinguished Postdoc Mentor Award. "I learned that the best strategy for me is to have a personalized approach. I give close to complete freedom to the best post-docs; others need more nurturing, especially at the beginning."

Sponsored by the University at Buffalo's Graduate School Office of Postdoctoral Scholars, the award was presented June 13 during the Postdoctoral Research Symposium.

"I am extremely honored by the award." says Feltri. "I feel it has true meaning because the nomination comes directly from my fellow co-workers.

Researchers ID Neuropathy Cause, Restore Myelination

Lawrence Wrabetz, MD.

Lawrence Wrabetz, MD

May 22, 2013

Neuroscientists at the University at Buffalo's Hunter James Kelly Research Institute (HJKRI) and European colleagues have provided proof of principle for how a genetic mutation leads to some neuropathies.

The international team also used a research drug to successfully alleviate the protein synthesis misstep, thereby improving myelin. Myelin is the fatty material that wraps the axons of neurons and allows them to signal effectively.

As a result, a potential new treatment strategy may be on the horizon for patients with a host of neurological disorders that result from misfolded proteins.

photo of untreated vs salubrinal treated culture from neuropathy mice.

New Research Suggests Treatment for Neurological Disorders such as CMT, Alzheimer's

April 29, 2013  

A potential new treatment strategy for patients with Charcot-Marie-Tooth disease is on the horizon, thanks to research by neuroscientists now at the University at Buffalo's Hunter James Kelly Research Institute and their colleagues in Italy and England.

The institute is the research arm of the Hunter's Hope Foundation, established in 1997 by Jim Kelly, Buffalo Bills Hall of Fame quarterback, and his wife, Jill, after their infant son Hunter was diagnosed with Krabbe Leukodystrophy, an inherited fatal disorder of the nervous system. Hunter died in 2005 at the age of eight. The institute conducts research on myelin and its related diseases with the goal of developing new ways of understanding and treating conditions such as Krabbe disease and other leukodystrophies.

Charcot-Marie-Tooth or CMT disease, which affects the peripheral nerves, is among the most common of hereditary neurological disorders; it is a disease of myelin and it results from misfolded proteins in cells that produce myelin.

Gustavo Della Flora Nunes

CURCA Funds Student's Biochemistry Research

February 28, 2013

An exchange student investigating the metabolic exchange that occurs between myelin and axons has received an award from the Center for Undergraduate Research and Creative Activities (CURCA).

Gustavo Della Flora Nunes, a scholar with Brazil's Science Without Borders program, conducts research with M. Laura Feltri, MD, professor of biochemistry and neurology.

Shedding Light on Axonal Damage Nunes' research could help scientists design protection for axons in demyelinating disorders like multiple sclerosis. He is studying what happens to axons when the metabolism of its surrounding glial cells is damaged. His research involves eliminating a crucial energy-producing component in myelinating glia. Myelin, made by glial cells, is the fatty sheath surrounding an axon, the part of a neuron that transmits impulses away from the cell body. Scientists believe that myelin cells provide nutrients and energy to neurons.

Sophie Belin, PhD

Postdoctoral Biochemist Awarded $40,000 Neuropathy Fellowship

September 5 , 2012

Sophie Belin, PhD, a postdoctoral research scientist in biochemistry, has received the 2012 Peripheral Nerve Society(PNS) Neuropathy Fellowship.

She was selected for the competitive $40,000 grant to investigate a pharmacological approach to treat hereditary neuropathy of the Charcot-Marie-Tooth (CMT) disease type.

Studying Arthritis Drug in Mouse Model of CMT Belin will work on the project with her mentor, Lawrence Wrabetz, MD, professor of neurology and biochemistry and director of the Hunter James Kelly Research Institute.

Arrival of Wrabetz and Feltri Moves Work of Kelly Institute Forward

(from left to right) Lawrence Wrabetz, MD and Laura Feltri, MD.

Lawrence Wrabetz, MD and Laura Feltri, MD

November 3, 2011  

The mission of the Hunter James Kelly Research Institute (HJKRI) is moving forward with great fervor with the recent arrival of highly regarded neuroscientists Lawrence Wrabetz and Laura Feltri.

Dubbed "physiccian-scientist superstars" by Michael Cain, dean of the School of Medicine and Biomedical Sciences, at the press conference in 2010 announcing the pair's recruitment to UB, Wrabetz is the institute's first director and holds a primary appointment in the Department of Neurology, with a secondary appointment in the Department of Biochemistry; Feltri has a primary appointment in Biochemistry.

The husband-wife research team arrived in Buffalo in late spring from Milan, Italy, with family and laboratories, including 10 research associates-eight of Italian and two of French descents-and 56 lines of transgenic mice.

(from left to right) Wrabetz, Feltri, Jill Kelly, Jim Kelly, Patricia Duffner and Michael Cain.

Attending a press conference to announce the appointments of Lawrence Wrabetz and Laura Feltri were, from left, Wrabetz, Feltri, Jill Kelly, Jim Kelly, Patricia Duffner and Michael Cain

Noted Researchers Join Kelly Institute

August 12, 2010

Lawrence Wrabetz, head of the myelin biology unit at San Raffaele Scientific Institute in Milan, Italy, has been appointed director of the Hunter James Kelly Research Institute (HJKRI) at UB.

Laura Feltri, who heads the neuroglia unit at the Italian institute and is Wrabetz's spouse, also has been recruited to the HJKRI, which was established in 2004 by UB and the Hunter's Hope Foundation. Wrabetz and Feltri will begin transitioning their laboratories to Buffalo this fall.

Both are highly regarded neuroscientists with significant backgrounds in basic and translational research on myelin, known as white matter-the sheath protecting brain nerve fibers that is essential for all normal functioning of the nervous system. They will work as a team in the HJKRI, located in UB's New York State Center of Excellence in Bioinformatics and Life Sciences on the Buffalo Niagara Medical Campus in downtown Buffalo.