Navigation Links
University of South Florida researchers find blood-brain barrier damaged by disease
Date:3/8/2011

A study into the effects of Sanfilippo Syndrome type B (MPS III B) has found that the barrier responsible for protecting the brain from the entry of harmful blood-borne substances is structurally and functionally damaged by the devastating disease. University of South Florida researchers identified damage in specific brain structures involved in the pathology of MPS III B, one of four Sanfilippo syndromes, all of which are inherited diseases of metabolism.

The study, using a mouse model of MPS III B, has been published online in the journal PLoS One. Before this study, little was known about the integrity of the blood-brain barrier in this disease. (www.plosone.com)

"These new findings about blood-brain barrier structural and functional impairment in MPS III B mice, even at early disease stage, may have implications for disease pathogenesis and should be considered in the development of treatments for MPS III B," said study lead author Svitlana Garbuzova-Davis, PhD, an assistant professor in the Department of Neurosurgery and Brain Repair at the University of South Florida.

Sanfilippo syndrome type B is caused by a deficiency in the Naglu gene, the gene responsible for producing an enzyme needed to degrade heparan sulfate. Naglu-deficient mice show progressive deterioration of movement, vision and hearing. Neurons in various parts of the brain including the olfactory bulb, cortex, thalamus, amygdala, and other areas are affected by the disease. Consequently, patients with MPS III B experience a variety of pathological brain changes.

"Among our findings was that endothelial cells and other cells comprising the blood-brain barrier are damaged, resulting in vascular leakage," said Dr. Garbuzova-Davis. "This compromise may lead to destruction of the fragile central nervous system equilibrium."

Dr. Garbuzova-Davis and her co-researchers also reported that the "insult to blood-brain barrier integrity" likely comes from accumulated storage products within the endothelial cells, which are primary cellular components of the BBB. The authors noted that it is possible that blood-brain barrier dysfunction occurred before, or concurrent with, the appearance of neuropathological changes in MPS III B.

"Interestingly, more capillary leakage was seen in some brain structures, such as the hippocampus, in early symptomatic mice than in late symptomatic mice," Dr. Garbuzova-Davis said. "We speculate that the regions of the brain differ in metabolic functional activity, especially in growing animals, and higher activity may require more substantial exchanges of nutrients and metabolic activity. If the blood-brain barrier is already weakened in these areas, more vascular leakage may occur."

The authors report that capillary endothelial cell dysfunction may accelerate neuropathological changes in MPS III B by potentially allowing harmful blood-borne soluble substances, including neurotoxins, to enter the central nervous system.

"Alternatively, damaged endothelial cells may alter specific mechanisms for transport of various solutes across the blood-brain barrier," said Paul Sanberg, PhD, DSc, executive director of USF Center of Excellence for Aging and Brain Repair and co-author of the paper. "In this scenario, neural cells might suffer the dual effects of reduced nutrition and increased metabolite levels, impairing central nervous system function."

The researchers concluded that determining the evolution of blood-brain barrier dysfunction in MPS III B is important for both understanding how the disease progresses and for developing therapies. One possibility for blood-brain barrier repair is replacement of affected endothelial cells with endothelial progenitor cells from bone marrow or umbilical cord blood. Because a microaneurysm was noted in the brain of a mouse modeling MPS III B, the authors also suggest that special attention be given to the possibility of cerebral hemorrhage in MPS III B patients caused by weakened integrity.


'/>"/>

Contact: Randolph Fillmore
rfillmor@health.usf.edu
813-974-0868
University of South Florida (USF Health)
Source:Eurekalert

Related medicine news :

1. University of Virginia Health System Medical Laboratories Selects Sunquest's Specimen Collection Solution
2. Akron Institute of Herzing University Launches Its First Bachelors Degree Programs to Prepare Students for Even Greater Success in Business, Health Care and IT
3. Most pandemic plans in Ontario hospitals have not been tested: Queens University study
4. San Diego State University and BIOCOM Institute Receive $4.95 Million Grant: The BRIDGE Project, Linking Education to Employment in San Diegos Life Sciences Industry
5. Smithsonian Institution, Arizona State University announce education and research partnership
6. Untreated poor vision in elderly linked to dementia, University of Michigan study shows
7. Giarmarco, Mullins & Horton, P.C. President Joseph F. Page to Speak at University of Michigan
8. Arizona State Universitys Decision Theater offers balance to an off-kilter world
9. Forest City Announces Joint Venture with Health Care REIT for University Park Life Science Properties
10. Herzing University Online Launches Master and Bachelor of Science Degrees in Nursing
11. Nurtur Acquires ActivHealth and Wellness by Choice; Gains Exclusive Partnership with Duke University Center for Living
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:4/22/2017)... ... April 22, 2017 , ... Ecommerce sales have grown every year ... be $394.9 billion. The consequences of rapid innovation and growth are often neglected ... technology, it is every business and individual’s job to give something back to the ...
(Date:4/21/2017)... ... 2017 , ... An April 10 article in the Daily Mail ... a great deal about prehistoric ice-age dental practitioners and their primitive and, no doubt, ... remove decayed dental matter, and that teeth were then filled with bitumen, a substance ...
(Date:4/21/2017)... SALT LAKE CITY, Utah (PRWEB) , ... April ... ... leading provider of wilderness therapy treatment for adolescents and young adults, has kicked ... Thursday, April 20th-Sunday, April 23rd. This year’s theme is “Attachment Informed Therapy ...
(Date:4/21/2017)... ... April 21, 2017 , ... The Patient Advocacy Community ... System, with the 2017 Ruth Ravich Patient Advocacy Award in recognition of ... DeVaro was honored with the award at The Beryl Institute’s annual Patient Experience ...
(Date:4/21/2017)... ... 21, 2017 , ... Alive for Wellness is a group of ... health struggles. The Alive team uses advanced behavioral sciences treatment modalities to accomplish ... a mental health struggle is based on 10 modalities of treatment that ...
Breaking Medicine News(10 mins):
(Date:4/19/2017)... -- Companion animal vaccines are ... such as canine, avian and feline. ... as Attenuated Live Vaccines, Conjugate Vaccines, Inactivated Vaccines, ... Vaccines. Attenuated live vaccines are derived from disease-causing ... been weakend under laboratory conditions. Conjugate vaccines are ...
(Date:4/18/2017)... Cogentix Medical, Inc. (NASDAQ: CGNT), a global ... and Gynecology markets with innovative and proprietary products, will ... 31, 2017 after the market close on Tuesday, May ... a conference call and webcast to discuss its financial ... at 4:30 p.m. Eastern Time (3:30 p.m. Central Time). ...
(Date:4/18/2017)... SUNNYVALE, Calif. , April 18, 2017 /PRNewswire/ ... results of collaboration started in 2016, in which ... "Artificial Brain SOINN". The companies achieved initial results ... ultrasound solution by Artificial Brain SOINN. The results ... Tokyo Big Sight, April 19-21, at booths 4505 ...
Breaking Medicine Technology: