Featured Research

from universities, journals, and other organizations

Mutations that cause Parkinson's disease prevent cells from destroying defective mitochondria

Date:
June 4, 2010
Source:
Rockefeller University Press
Summary:
Mutations that cause Parkinson's disease prevent cells from destroying defective mitochondria, according to a new study.

Mutations that cause Parkinson's disease prevent cells from destroying defective mitochondria, according to a study published online May 10 in the Journal of Cell Biology.

Defects in the ubiquitin ligase Parkin are linked to early-onset cases of this neurodegenerative disorder. The wild-type protein promotes the removal of impaired mitochondria by a specialized version of the autophagy pathway called mitophagy, delivering mitochondria to the lysosomes for degradation. Mitochondria are often dysfunctional in Parkinson's disease, but how Parkin stimulates mitophagy and whether the pathway goes wrong during pathogenesis is unknown.

A team of researchers led by Tso-Pang Yao (Duke University) found that cells expressing mutant forms of Parkin failed to clear their mitochondria after the organelles were damaged. Different mutations blocked mitophagy at distinct steps: mitochondria accumulated in the perinuclear region of cells expressing Parkin lacking its ubiquitin ligase activity, for example. The researchers found that ubiquitination of defective mitochondria by Parkin normally recruits the autophagy proteins HDAC6 and p62 to clear these mitochondrial aggregates.

Depolymerizing microtubules or inhibiting the dynein motor protein blocked aggregation and prevented mitochondrial turnover. Transport to the perinuclear region was also blocked by a mutation in Parkin, indicating that this stage of mitophagy is also regulated by the protein.

The clearance of defective mitochondria is therefore similar to the removal of damaged proteins, another autophagic process that goes wrong in Parkinson's disease resulting in the accumulation of toxic protein aggregates. Both pathways rely on microtubules, HDAC6, and p62, says Yao, providing a common link between the two main features of the neurodegenerative disorder.


Story Source:

The above story is based on materials provided by Rockefeller University Press. Note: Materials may be edited for content and length.


Journal Reference:

  1. Joo-Yong Lee, Yoshito Nagano, J. Paul Taylor, Kah Leong Lim, Tso-Pang Yao. Disease-causing mutations in Parkin impair mitochondrial ubiquitination, aggregation, and HDAC6-dependent mitophagy. Journal of Cell Biology, 2010; DOI: 10.1083/jcb.201001039

Cite This Page:

Rockefeller University Press. "Mutations that cause Parkinson's disease prevent cells from destroying defective mitochondria." ScienceDaily. ScienceDaily, 4 June 2010. <www.sciencedaily.com/releases/2010/05/100510121215.htm>.
Rockefeller University Press. (2010, June 4). Mutations that cause Parkinson's disease prevent cells from destroying defective mitochondria. ScienceDaily. Retrieved September 21, 2014 from www.sciencedaily.com/releases/2010/05/100510121215.htm
Rockefeller University Press. "Mutations that cause Parkinson's disease prevent cells from destroying defective mitochondria." ScienceDaily. www.sciencedaily.com/releases/2010/05/100510121215.htm (accessed September 21, 2014).

Share This



More Mind & Brain News

Sunday, September 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Could Grief Affect The Immune Systems Of Senior Citizens?

Could Grief Affect The Immune Systems Of Senior Citizens?

Newsy (Sep. 19, 2014) The study found elderly people are much more likely to become susceptible to infection than younger adults going though a similar situation. Video provided by Newsy
Powered by NewsLook.com
Food Addiction Might Be Caused By PTSD

Food Addiction Might Be Caused By PTSD

Newsy (Sep. 18, 2014) New research shows that women who suffer from PTSD are three times more likely to develop a food addiction. Video provided by Newsy
Powered by NewsLook.com
Corporal Punishment on Decline, Debate Renews

Corporal Punishment on Decline, Debate Renews

AP (Sep. 16, 2014) Corporal punishment in the United States is on the decline, but there is renewed debate over its use after Minnesota Vikings running back Adrian Peterson was charged with child abuse. (Sept. 16) Video provided by AP
Powered by NewsLook.com
FDA Eyes Skin Shocks Used at Mass. School

FDA Eyes Skin Shocks Used at Mass. School

AP (Sep. 15, 2014) The FDA is considering whether to ban devices used by the Judge Rotenberg Educational Center in Canton, Massachusetts, the only place in the country known to use electrical skin shocks as aversive conditioning for aggressive patients. (Sept. 15) Video provided by AP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:
from the past week

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins