Navigation Links
Neurological disorder impacts brain cells differently
Date:11/9/2011

In a paper published in the Nov. 9 issue of the Journal of Neuroscience, researchers at the University of California, San Diego School of Medicine and University of Washington describe in deeper detail the pathology of a devastating neurological disorder, but also reveal new cellular targets for possibly slowing its development.

Spinocerebellar ataxia type 7 (SCA7) is an inherited neurological disorder in which cells in the cerebellum and brainstem degenerate, resulting in progressive loss of physical coordination and possible blindness. Its pathology is similar to other neurodegenerative diseases like Parkinson's, Huntington's and amyotrophic lateral sclerosis. And like them, it's currently incurable.

The scientists, led by Al La Spada, MD, PhD, chief of the division of genetics in the UC San Diego department of pediatrics, and professor of cellular and molecular medicine, neurosciences and biological sciences, used a variety of transgenic mouse models to show that SCA7 results from genetic dysfunction not just in affected neurons, but also in associated non-neuronal support cells.

"The central nervous system is quite complicated, with neurons interacting with each other and with other cell types. So it shouldn't be a surprise that the disease process is similarly complex," said La Spada, who is also associate director of the UC San Diego Institute for Genomic Medicine. "We show that dysfunction in a variety of cell types contributes to SCA7, and that if you can improve function in any of these cell types, you have a reasonable chance of improving treatment of the disease."

La Spada and colleagues created a transgenic mouse in which the key gene mutation that causes SCA7 could be easily manipulated. The mouse was then bred with other mouse models that eliminated the mutant gene protein from specific cell types affected by SCA7: Purkinje neurons (large cells in the cerebral cortex responsible for motor coordination), Bergmann glia (support cells found in the cerebellum) and cells in the olivary complex (part of the brainstem controlling body movement).

By creating and comparing mice that expressed the mutant gene only in targeted cells, La Spada said the scientists made two unexpected discoveries: First, when the gene mutation was eliminated from Bergmann glia, neurodegeneration continued unabated and still involved dysfunction and degeneration of the Bergmann glia themselves. Second, when the mutation was excised from Purkinje neurons and the olivary complex, there was significantly less neurological damage and Bergmann glia remained intact.

"The first result highlights the relatively new idea that degeneration goes both ways," said La Spada. "It isn't just neurons becoming affected when their support cells dysfunction. The Bergmann glia didn't express the mutant gene, but they still degenerated. This shows the bilateral relationship between neurons and non-neuronal cells. They're equal partners, in both normal functioning and in disease.

"The second result underscores the relevance of Purkinje cells and the olivary neuron circuit in the brainstem to SCA7. When it's dysfunctional, degeneration occurs. This is crucial for our understanding of this disease, and should enable us to develop more specific therapeutic approaches. Although we have our work cut out for us, we now have a better idea of what we're up against."


'/>"/>
Contact: Scott LaFee
slafee@ucsd.edu
619-543-6163
University of California - San Diego
Source:Eurekalert  

Related medicine news :

1. Harvard Medical School and EPFL launch program targeting neurological disabilities
2. Researcher tests drugs impact on neurological disease affecting women
3. Cedars-Sinai to hold first annual conference on stem cell therapies for neurological disorders
4. Neurological protein may hold the key to new treatments for depression
5. Elseviers BrainNavigator 3.0 reflects the next step in enhancing neurological research
6. NYU researchers identify new neurological deficit behind lazy eye
7. Scientists explain the neurological process for the recognition of letters and numbers
8. Team led by LA BioMed scientist develops novel approach to study neurological disorders
9. University Hospitals Neurological Institute earns Neuroscience Center of Excellence designation
10. University Hospitals Neurological Institute hosts international epilepsy colloquium
11. Rare disease in Amish children sheds light on common neurological disorders
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Neurological disorder impacts brain cells differently
(Date:12/7/2016)... ... ... They are musicians and librarians, fashion designers and fitness instructors, actors, athletes ... and around the nation. What do they have in common? All have been affected ... compelling new photographic exhibit debuting Friday, December 9 at Logan International Airport in Boston. ...
(Date:12/7/2016)... ... December 07, 2016 , ... ODU, ... promoting to the US market its advanced highly customizable contact technology solutions. , ... TURNTAC®. These advanced technologies are ideal for a wide range of applications that ...
(Date:12/7/2016)... ... December 07, 2016 , ... Baciocco Brothers ... to residents in the Sacramento/Folsom region, is initiating a charity event to raise ... Another Choice Another Chance treatment center in Sacramento works to provide area teens ...
(Date:12/7/2016)... , ... December 07, 2016 , ... One of two ... of the securement tape is painful for her. "This is why the co-inventor and ... heads," she said. , They then created a prototype of the patent-pending AV-AIR, a ...
(Date:12/7/2016)... ... December 07, 2016 , ... A. Kevin Spann Insurance, a ... throughout the Five Boroughs, is launching a charity drive to raise funds that will ... traditions and spirit of marines and Navy FMF Corpsmen. Working closely with the MCL, ...
Breaking Medicine News(10 mins):
(Date:12/7/2016)... KEY FINDINGS Patient warming ... reducing loss of blood during surgeries, lowering the ... recovery after surgeries, and decreasing risks of SSIs. ... convective warming system, surface warming systems, and intravascular ... stay at hospitals thus, lowering the healthcare costs ...
(Date:12/7/2016)... 7, 2016 Global Hospital Linen Supply and ... global hospital linen supply and management services market presents ... a global and regional level. The study provides historic ... between 2015 and 2024 based on revenue (US$ Bn). ... market along with their impact on demand during the ...
(Date:12/7/2016)... N.J. , Dec. 7, 2016  Palatin ... it has closed on a previously disclosed underwritten ... $16,500,000.  Canaccord Genuity acted as sole book-running manager, ... Chardan Capital Markets acted as co-manager for the ... approximately $15.4 million in net proceeds, allowing us ...
Breaking Medicine Technology: