New! Sign up for our free email newsletter.
Science News
from research organizations

New structural insight into neurodegenerative disease

Date:
March 14, 2013
Source:
The Korea Advanced Institute of Science and Technology (KAIST)
Summary:
Scientists have released new results on the structure and molecular details of the neurodegenerative disease-associated protein Ataxin-1. Mutations in Ataxin-1 cause the neurological disease, Spinocerebella Ataxia Type 1, which is characterized by a loss of muscular coordination and balance, as is seen in Parkinson's, Alzheimer's, and Huntington's diseases.
Share:
FULL STORY

A research team from the Korea Advanced Institute of Science and Technology (KAIST) released their results on the structure and molecular details of the neurodegenerative disease-associated protein Ataxin-1. Mutations in Ataxin-1 cause the neurological disease, Spinocerebella Ataxia Type 1 (SCA1), which is characterized by a loss of muscular coordination and balance (ataxia), as is seen in Parkinson's, Alzheimer's, and Huntington's diseases.

SCA1-causing mutations in the ATAXIN1 gene alter the length of a glutamine stretch in the Ataxin-1 protein. The research team provides the first structural insight into the complex formation of ATAXIN-1 with its binding partner, Capicua (CIC). The team, led by Professor Ji-Joon Song from the Department of Biological Sciences at KAIST, solved the structure of Ataxin-1 and CIC complex in atomic level revealing molecular details of the interaction between Ataxin-1 and CIC.

Professor Song explained his recent research work, "We are able to see the intricate process of complex formation and reconfiguration of the two proteins when they interact with each other. Our work, we expect, will provide a new therapeutic target to modulate SCA1 neurodegenerative disease."

Understanding structural and molecular details of proteins at the atomic level will help researchers to track the molecular pathogenesis of the disease and, ultimately, design targeted therapies or treatments for patients, rather than just relieving the symptoms of diseases.


Story Source:

Materials provided by The Korea Advanced Institute of Science and Technology (KAIST). Note: Content may be edited for style and length.


Journal Reference:

  1. Eunji Kim, Hsiang-Chih Lu, Huda Y. Zoghbi, and Ji-Joon Song. Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein ATAXIN-1 and Capicua. Genes and Development, Mar 15, 2013 (in press)

Cite This Page:

The Korea Advanced Institute of Science and Technology (KAIST). "New structural insight into neurodegenerative disease." ScienceDaily. ScienceDaily, 14 March 2013. <www.sciencedaily.com/releases/2013/03/130314180142.htm>.
The Korea Advanced Institute of Science and Technology (KAIST). (2013, March 14). New structural insight into neurodegenerative disease. ScienceDaily. Retrieved April 17, 2024 from www.sciencedaily.com/releases/2013/03/130314180142.htm
The Korea Advanced Institute of Science and Technology (KAIST). "New structural insight into neurodegenerative disease." ScienceDaily. www.sciencedaily.com/releases/2013/03/130314180142.htm (accessed April 17, 2024).

Explore More

from ScienceDaily

RELATED STORIES