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.
The preclinical research, published in the Journal of Neuroscience, concerns oligodendrocytes, the cells that make myelin, and the progenitor cells that are their precursors.
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.
Fifteen of UBï¿½s best and brightest teachers and researchers have been named recipients of the universityï¿½s 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.
The Exceptional Scholars Award honors faculty members for their outstanding research performance at different stages of their careers. There are two awards: Sustained Achievement Awards for senior scholars and Young Investigator Awards for untenured scholars who received their terminal degree within the past eight years. Both awards recognize work that has ï¿½garnered public and/or professional accolades beyond the norm.ï¿½
Sustained Achievement recipients are selected based on their body of work over a number of years. The award is not meant to serve as a lifetime achievement honor, but rather as recognition for outstanding performance in a recent segment of a scholarï¿½s career.
Recipients for 2016 are Brahm Segal, professor, Department of Medicine; Irus Braverman, professor, School of Law; Thomas Feeley, professor, Department of Communication; Doreen Wackeroth, professor, Department of Physics; M. Laura Feltri, professor, departments of Biochemistry and Neurology; Murali Ramanathan, professor, departments of Pharmaceutical Sciences and Neurology; and Kui Ren, professor, Department of Computer Science and Engineering.
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. ï¿½We know ... that after a bone fracture, one should put weight on the broken bone because this mechanical stimulation improves the formation of new bone cells. Now we know that a similar phenomenon is occurring with myelin cells.ï¿½ Their findings, published online June 6 in Nature Neuroscience, may signal a path to new therapies for multiple sclerosis and other myelin-related diseases.
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.
"This federal investment will support exceptional research happening right here at the Hunter James Kelly Research Institute that is providing promising advancements toward achieving better detection and treatment for Krabbe disease," Higgins said.
Krabbe disease is diagnosed through a series of tests, which often includes a blood test to determine the level of GALC enzyme activity. Other tests can include a MRI, CT, nerve conduction study and genetic testing for mutation analysis. Effective newborn screening can lead to correct and early diagnosis and treatment. Patients diagnosed before the disease is too far progressed may be eligible for a cord blood transplant, which can halt the progress of the disease.
"This grant to the Hunter James Kelly Research Institute is aimed at improving diagnosis of Krabbe disease by newborn screening," said Thomas J. Langan, M.D., principal investigator on the grant, clinical director of the Hunter James Kelly Research Institute, and associate professor of neurology, pediatrics and physiology and biophysics at the Jacobs School of Medicine and Biomedical Sciences at UB. "One of the biggest challenges with newborn screening of Krabbe's disease is false positives, test results that suggest that a disease is present, when it actually is not."
Myelination depends on mechanical as well as chemical signaling and that may signal a new path to treatments for myelin diseases, such as MS.
BUFFALO, N.Y. ï¿½ 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 paper was published online June 6 in Nature Neuroscience.
The authors are scientists at UBï¿½s Hunter James Kelly Research Institute (HJKRI), one of a handful of research institutes in the world with an exclusive focus on myelin and diseases of myelin, such as multiple sclerosis and leukodystrophies, and how they may be treated. The institute, part of UBï¿½s New York State Center of Excellence in Bioinformatics and Life Sciences, 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 Krabbe Leukodystrophy, an inherited, fatal disorder. He died in 2005 at the age of 8.
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 University at Buffaloï¿½s Medical Scientist Training Program (MD/PhD) has been named a local chapter of the American Physician Scientists Association (APSA).
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.
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.
UB researcher contributes to study on new candidate drug that may help treat misfolded protein diseases like CMT, Alzheimerï¿½s and Parkinsonï¿½s.
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.
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.
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.
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.
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.
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."
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.Continue reading... |
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.Continue reading... |
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.Continue reading... |
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.Continue reading... |
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.Continue reading... |
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.Continue reading... |