Navigation Links
Defective protein is a double hit for ataxia
Date:4/5/2010

The neurodegenerative disease spinocerebellar ataxia type 5 (SCA5) damages nerve cells in two ways. University of Minnesota researchers now report that the defective protein responsible for the disease cuts the number of synaptic terminals and snarls traffic inside neurons. The study appears in the April 5 issue of the Journal of Cell Biology (www.jcb.org).

SCA5 results from a faulty gene for {beta}-IIIspectrin. The disease targets the cerebellum's Purkinje cells, which control coordination. Symptoms usually start when patients are in their 20s or 30s, and they can gradually lose the ability to walk and speak. How the mutant protein damages Purkinje cells remains uncertain. {beta}-IIIspectrin stabilizes synapses, suggesting that synapse deterioration might doom the cells. But the protein also helps the adapter protein dynactin hitch cargoes to dynein motors, pointing to a disruption of intracellular transportation.

Lorenzo et al. found support for both mechanisms. They engineered fruit flies to carry a mutated {beta}-III spectrin gene from either of two human families affected with SCA5, including one descended from Abraham Lincoln's paternal grandparents (whether Lincoln himself had the disease isn't clear). Fly larvae with the mutated gene had paralyzed tails. At the neuromuscular junctions where nerves and muscles meet, the larvae showed fewer presynaptic terminals.

The researchers next tracked the movement of synaptic vesicles in axons from the animals. Vesicles from flies that made the faulty {beta}-IIIspectrin were slower and more likely to change direction, and thus traveled shorter distances. Other neurodegenerative diseases, including Alzheimer's disease and amyotrophic lateral sclerosis, involve faulty transport, and the results indicate that SCA5 does too.

The two mechanisms might have a common link, the researchers suggest. The complex containing {beta}-IIIspectrin, dynactin, and dynein might not just haul cargoes. At the synapse it might snag microtubules that strengthen the membrane and prevent degeneration.


'/>"/>

Contact: Rita Sullivan
news@rupress.org
212-327-8603
Rockefeller University Press
Source:Eurekalert  

Related biology news :

1. Research project yields better understanding of the defective protein that causes cystic fibrosis
2. Dangerous plaques in blood vessels rupture by overproducing protein-busting enzymes
3. Discovery that PARP protein exists in all breast tumors will help target chemo and predict response
4. New understanding of proteins role in brain
5. Key enzyme discovered to be master regulator in protein-protein reactions
6. Colonies of bacteria fight for resources with lethal protein
7. Sea creatures sex protein provides new insight into diabetes
8. Duke technique is turning proteins into glass
9. Measuring protein movements with nanosecond resolution
10. Light activated warhead turns modest molecules into super protein killers
11. Behavior of single protein observed in unprecedented detail by Stanford chemists
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Defective protein is a double hit for ataxia
(Date:1/24/2017)... , Jan. 24, 2017  It sounds simple ... sock that monitors vital signs and alerts parents ... infant,s oxygen saturation level drops. But pediatric experts ... to parents, with no evidence of medical benefits, ... are marketed aggressively to parents of healthy babies, ...
(Date:1/21/2017)... , Jan 20, 2017 Research and ... Biometrics Market 2017-2021" report to their offering. ... The global voice recognition biometrics ... 2017-2021. The report covers the present scenario and ... 2017-2021. To calculate the market size, the report considers the revenue ...
(Date:1/19/2017)... SANTA CLARA, Calif. , Jan. 19, 2017 ... Valley company enhancing user experience and security for ... provider of next-generation payment processing systems and cybersecurity ... assist more banks, enterprises and financial institutions worldwide ... As part of the end-to-end secure user authentication ...
Breaking Biology News(10 mins):
(Date:2/23/2017)... ... February 23, 2017 , ... Brain Sentinel, Inc. ... marketing the SPEAC® System, the Brain Sentinel® Seizure Monitoring and Alerting System. The ... facilities during periods of rest. A lightweight, non-invasive monitor is placed on the ...
(Date:2/23/2017)... 2017  Capricor Therapeutics, Inc. (NASDAQ: CAPR), a biotechnology company ... announced that Linda Marbán, Ph.D, president and chief executive officer, ... Cowen and Company 37th Annual Health Care ... Boston, MA 29th Annual ... (12:00 pm ET) Dana Point, CA ...
(Date:2/22/2017)... ... February 22, 2017 , ... ... acquired Kendall Research Systems, LLC (KRS) clinical development program. KRS is ... interface technology for research and clinical applications. The terms of the transaction were ...
(Date:2/22/2017)... ON (PRWEB) , ... February 22, 2017 , ... ... on discovery and development of precision treatments for neurodegenerative diseases, today announced it ... validate the ProMIS approach.” This is one of a series of commentaries from ...
Breaking Biology Technology: