November 10, 2022
This year University at Buffalo, Society of Neuroscience chapter conducted an annual research day. Along with oral presentations, many students participated in poster presentations. Out of 47 poster presentations, Sri Laxmi Veerapaneni is one of the four students who received the award. For abstracts and details, visit: https://sfn.med.buffalo.edu
Sri Laxmi Veerapaneni, a graduate student works in the lab of M. Laura Feltri, MD, SUNY Distinguished Professor of biochemistry and neurology and director of the Institute for Myelin and Glia Exploration.
She is studying the role of Transcription factor EB (TFEB) in peripheral myelination. Transcription factor EB (TFEB) is a master transcriptional factor of autophagy and lysosomal proteins. Aside from its role in lysosomal debris clearance and autophagy, recent studies have shown that hyperactive TFEB in oligodendrocytes can repress myelination, and its absence can cause ectopic myelination during development in the central nervous system (CNS). She hypothesizes that TFEB has a similar role in the peripheral nervous system (PNS) which might help in further understanding of demyelinating diseases.
March 11, 2022
The two Jacobs School students to receive a 2022 SUNY GREAT Award are Steven A. Lewis, a trainee in the doctoral program in computational cell biology, anatomy and pathology; and Narayan Dhimal, a trainee in the doctoral program in neuroscience.
They are among 28 SUNY students receiving the GREAT awards; all award winners have also been recognized nationally for their research by prestigious graduate fellowship programs sponsored by the National Science Foundation (NSF) or the National Institutes of Health (NIH).
He is studying the roles of autophagy and TFEB transcription factor in Krabbe disease (KD), a demyelinating disorder of the central and the peripheral nervous system caused by lack of GALC (galactosylceramidase)
From his work, Dhimal says he hopes to find a therapeutic strategy where pharmacologically increasing or modulating the autophagy process might be beneficial and may serve as a combination treatment, along with bone marrow therapy, as well as ongoing gene therapy trials.
March 8, 2022
A few of the women at the Institute for Myelin and Glia Exploration got together to celebrate #IWD2022 by striking a pose in support the goals of this day and posting on social media:
The 5 women in this photo hail from 4 continents: Asia; Europe; South America and North America.
For more information see: https://www.internationalwomensday.com
Chancellor Malatras Announces the Winners of the Inaugural Chancellor Distinguished PhD Graduate Disseration Awards.
Honorable mention includes:
Albany, NY – State University of New York Chancellor Jim Malatras today announced winners of the inaugural Chancellor Distinguished PhD Graduate Dissertation Awards. Frist place was awarded to Binghamton University, Department of History student Dr. Jonathan S. Jones for his dissertation, “Opium Slavery: Veterans and Addiction in the American Civil War Era.” Jones and four finalists were selected amongst nominations of SUNY PhD student dissertations. Jones received the top prize of $5,000 to be used for professional development, and finalists were awarded $1,000 for professional development opportunities as well. The three honorable mentions were awarded $250. Each awardee’s work will be published by SUNY Press—a rare opportunity in public higher education—in Spring 2022.
May 7, 2021
Two UB drug development and discovery projects have received investments from the Empire Discovery Institute.
One project focuses on an immunotherapy platform — developed by UB pharmacy researcher Sathy Balu-Iyer and his team — that could benefit patients who are receiving gene therapy for a variety of diseases. The other will seek to design a therapy for multiple sclerosis (MS) that leverages a novel drug target discovered by a team led by UB medical researcher M. Laura Feltri.
The institute will advance both efforts through its Medicines Discovery Award Program, a competitive incubator and accelerator program designed to identify and advance promising early-stage drug discovery into early “proofs of concept.” Successful projects will exit the program as either a licensing transaction to a strategic pharmaceutical partner or as an Empire Discovery Institute-created startup company.
Empire Discovery Institute is a nonprofit New York drug discovery and development accelerator created to translate important academic discoveries into new medicines for commercialization by providing strategic support and pharmaceutical development expertise. Established with $35.4 million in initial funding from Empire State Development, it works in partnership with research labs at UB, the University of Rochester and Roswell Park Comprehensive Cancer Center.
February 3, 2021
Forty-three faculty members with a variety of clinical and research experience — representing 12 medical school departments — have joined the Jacobs School of Medicine and Biomedical Sciences over the past several months.
Daesung Shin, PhD, is an assistant professor of biotechnical and clinical laboratory sciences. He has expertise in areas including gene expression, gene therapy, inherited metabolic disorders, molecular and cellular biology, molecular genetics, neurobiology, signal transduction, and transgenic organisms.
Shin earned his doctoral degree from the Korea Advanced Institute of Science and Technology. He comes to the Jacobs School from the Hunter James Kelly Research Institute.
January 29, 2021
Students and laboratories affiliated with the Genetics, Genomics and Bioinformatics graduate program (GGB) shared their findings during the seventh annual GGB Research Day.
There were 14 participants, with four delivering oral presentations and 10 presenting a research poster. Cash prizes of $100 each were awarded to the best in category in oral presentation and poster presentation.
The “Best Poster Presentation Award” was presented to Narayan Dhimal, a doctoral student in the neuroscience program, for his study titled “Autophagy is Defective in Schwann Cells in Krabbe Disease.”
October 2, 2020
New research reveals for the first time that despite the fragility of axons, Schwann cells — which surround axons within nerves like a glove covers a hand — can come to the assistance of injured axons.
Axons are long, finger-like projections of neurons that transmit critical signals throughout the nervous system. But because they are energetically difficult to maintain, they are often among the first casualties of certain neurodegenerative diseases, causing symptoms such as muscle weakness or numbness of limbs.
The paper, “A Glycolytic Shift in Schwann Cells Supports Injured Axons,” was published in Nature Neuroscience.
August 25, 2020
Axons are long, finger-like projections of neurons that transmit critical signals throughout the nervous system. But because they are fragile, they are often among the first casualties of certain neurodegenerative diseases, causing symptoms such as muscle weakness or numbness of limbs.
New research from the University at Buffalo reveals that despite the fragility of axons, the cellular envelope (Schwann cells) that encases them can come to their rescue when it senses that axons have been harmed.
Published in Nature Neuroscience last week, the paper reveals for the first time that Schwann cells, which surround axons within nerves like a glove covers a hand, can come to the assistance of injured axons.
July 8, 2020
Jacobs School of Medicine and Biomedical Sciences researchers have published a paper that clarifies certain cellular mechanisms that could lead to improved outcomes in patients with globoid cell leukodystrophy, commonly known as Krabbe disease.
The paper, titled “Macrophages Expressing GALC Improve Peripheral Krabbe Disease by a Mechanism Independent of Cross-Correction,” was featured as the cover story published July 8 in the journal Neuron, with a “preview” synopsis written by Vittorio Gallo, PhD, chief research officer of the Children’s National Hospital and scientific director of the Children’s National Research Institute.
The research was led by M. Laura Feltri, MD, and Lawrence Wrabetz, MD, co-directors of the Hunter James Kelly Research institute (HJKRI) and professors of biochemistry and neurology in the Jacobs School; and by Daesung Shin, PhD, research assistant professor of biochemistry.
May 5, 2020
BUFFALO, N.Y. – A group of researchers at the University at Buffalo have published a paper that clarifies certain cellular mechanisms that could lead to improved outcomes in patients with globoid cell leukodystrophy, commonly known as Krabbe disease.
The paper, titled “Macrophages Expressing GALC Improve Peripheral Krabbe Disease by a Mechanism Independent of Cross-Correction,” was published today (May 5) in the journal Neuron.
The research was led by Lawrence Wrabetz, MD, and M. Laura Feltri, MD. Wrabetz and Feltri head the Hunter Jameas Kelly Research Institute (HJKRI) and both are professors in the departments of Biochemistry and Neurology in the Jacobs School of Medicine and Biomedical Sciences at UB.
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 (HJKRI), 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.”
April 9, 2020
Five UB faculty members have been named SUNY Distinguished Professors, the highest faculty rank in the SUNY system.
M. Laura Feltri, Jo Freudenheim, Amit Goyal, Elad Levy and Stephen Tiffany were appointed to the distinguished professor ranks by the SUNY Board of Trustees at its meeting on March 17.
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.
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."
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.
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.
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 (HJKRI) 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.
October 25, 2018
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 Institute.
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’
Elisabetta Babetto, PhD, senior research scientist in biochemistry and research assistant professor of pharmacology and toxicology in the Institute, was awarded a grant funded by GBS/CIDP Foundation International.
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.”
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:
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.
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.
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.
May 10, 2017
A 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]
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.
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.
Weinstock’s presentation was titled “Understanding Krabbe Disease.”
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.
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.
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.
November 14, 2016
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.
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
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.
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.
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.
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.
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.
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
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.
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.
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” ...
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.
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.
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.
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.
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.
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.
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.
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.
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).
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.
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.
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.
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.