First Mouse Model for Multiple System Atrophy
Mar 24, 2005 - 7:20:00 PM
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"The uniqueness of this disease is that, unlike most of the neurodegenerative diseases, which affect neurons primarily and oligodendrocytes secondarily, this is the other way around. In fact, there is growing evidence that non-neuronal cells also play a role in amyloid deposits in Alzheimer's disease and amyotrophic lateral sclerosis (ALS) mouse models"
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By University of Pennsylvania Medical Center,
[RxPG] A newly developed animal model for Multiple System Atrophy (MSA) a collection of neurodegenerative disorders once thought to be three separate diseases sheds new light on this little-studied brain disease, according to research from investigators at the University of Pennsylvania School of Medicine.
Virginia M.-Y. Lee, PhD, Director of Penn's Center for Neurodegenerative Disease Research, and colleagues demonstrated that the mice showed symptoms similar to human MSA. These include an accumulation of a protein called á-synuclein in oligodendrocytes cells that produce the protective myelin sheath that covers axons. This protein accumulation disables oligodendrocytes, leading to a loss of the sheath on neurons and eventually nerve-cell malfunction and death. The mice also showed slowly progressive problems with their motor skills associated with the nerve-cell damage. Neurons are important in transmitting signals and in maintaining learning and memory.
"The uniqueness of this disease is that, unlike most of the neurodegenerative diseases, which affect neurons primarily and oligodendrocytes secondarily, this is the other way around," says Lee. In fact, there is growing evidence that non-neuronal cells also play a role in amyloid deposits in Alzheimer's disease and amyotrophic lateral sclerosis (ALS) mouse models. Lee and colleagues report their findings in the March 24, 2005 issue of Neuron.
MSA is so named because it affects multiple parts of the nervous system. Initially MSA was given three names, based on the symptoms physicians had observed. However, when they closely examined patients' pathology, the disorders seemed related, based on the á-synuclein proteins in cells. In the clinic, many patients with MSA present with symptoms similar to Parkinson's disease (PD), and MSA has been misdiagnosed as such.
Collectively, MSA now includes three related disorders characterized by their most prominent symptoms: olivopontocerebellar atrophy, which affects balance, coordination, and speech; striatonigral degeneration, the closest to Parkinson's disease because of slow movement and stiff muscles; and Shy-Drager syndrome, which involves altered bowel, bladder, and blood-pressure control. Other general symptoms include dizziness, impaired speech, breathing and swallowing difficulties, and blurred vision. Most patients develop dementia late in the course of the disease, which is usually diagnosed in people over 50.
Currently there is no specific drug to treat the myelin and nerve damage caused by the protein inclusions. Parkinson's disease drugs and others are used to alleviate early symptoms. "With this animal model, we now can plan tests of potential therapies for Multiple System Atrophy as part of our drug discovery program for Parkinson's disease, MSA, and related disorders," says Lee.
Publication:
Lee and colleagues report their findings in the March 24, 2005 issue of Neuron.
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Additional information about the news article
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The study was funded in part by the National Institutes of Health. Ikuru Yazawa, Benoit I. Giasson, Ryogen Sasaki, Bin Zhang, Sonali Joyce, Kunihuro Uryu, and John Q. Trojanowski, all from Penn, are study co-authors. The authors report no conflicts of interests related to this research.
PENN Medicine is a $2.7 billion enterprise dedicated to the related missions of medical education, biomedical research, and high-quality patient care. PENN Medicine consists of the University of Pennsylvania School of Medicine (founded in 1765 as the nation's first medical school) and the University of Pennsylvania Health System.
Penn's School of Medicine is ranked #3 in the nation for receipt of NIH research funds; and ranked #4 in the nation in U.S. News & World Report's most recent ranking of top research-oriented medical schools. Supporting 1,400 fulltime faculty and 700 students, the School of Medicine is recognized worldwide for its superior education and training of the next generation of physician-scientists and leaders of academic medicine.
The University of Pennsylvania Health System includes three owned hospitals [Hospital of the University of Pennsylvania, which is consistently ranked one of the nation's few "Honor Roll" hospitals by U.S. News & World Report; Pennsylvania Hospital, the nation's first hospital; and Presbyterian Medical Center]; a faculty practice plan; a primary-care provider network; two multispecialty satellite facilities; and home care and hospice
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