Navigation Links
Quality control of mitochondria as a defense against disease

HEIDELBERG, 20 January 2014 Scientists from the Montreal Neurological Institute and Hospital in Canada have discovered that two genes linked to hereditary Parkinson's disease are involved in the early-stage quality control of mitochondria. The protective mechanism, which is reported in The EMBO Journal, removes damaged proteins that arise from oxidative stress from mitochondria.

"PINK1 and parkin, are implicated in selectively targeting dysfunctional components of mitochondria to the lysosome under conditions of excessive oxidative damage within the organelle," said Edward Fon, Professor at the McGill Parkinson Program at the Montreal Neurological Institute and Hospital. "Our study reveals a quality control mechanism where vesicles bud off from mitochondria and proceed to the lysosome for degradation. This method is distinct from the degradation pathway for damaged whole mitochondria which has been known for some time. It is also an early response, proceeding on a timescale of hours instead of days."

The deterioration of mechanisms designed to maintain the integrity and function of mito-chondria throughout the lifetime of a cell has been suggested to underlie the progression of several neurodegenerative diseases, including Parkinson's disease. When mitochon-dria, the "power plants" of the cell that provide energy, malfunction they can contribute to Parkinson's disease. If they are to survive and function mitochondria need to degrade oxidized and damaged proteins.

In the study, immunofluorescence and confocal microscopy were used to observe how the vesicles "pinch off" from mitochondria with their damaged cargo. "Our conclusion is that the loss of this PINK1 and parkin-dependent trafficking system impairs the ability of mitochondria to selectively degrade oxidized and damaged proteins and leads, over time, to the mitochondrial dysfunction noted in hereditary Parkinson's disease," said Hei-di McBride, Professor in the Neuromuscular Group in the Department of Neurology and Neurosurgery at the Montreal Neurological Institute and Hospital. Both salvage pathways are operational in the cell. If the vesicular pathway, the first line of defense, is overwhelmed and the damage is irreversible then the entire organelle is targeted for degradation.


Contact: Barry Whyte
European Molecular Biology Organization

Related biology news :

1. Updating air pollution measurement methods with air quality, health effects research
2. Testosterone in male songbirds may enhance desire to sing but not song quality
3. Harvard study shows sprawl threatens water quality, climate protection, and land conservation gains
4. Quality of biodiversity, not just quantity, is key
5. Zinc sulfate, sugar alcohol zinc sprays improve apple quality
6. Protein quality: It matters
7. Enrollment in SNAP does not substantially improve food security or dietary quality
8. Clean Air Act has led to improved water quality in the Chesapeake Bay watershed
9. Laser technology sorting method can improve Capsicum pepper seed quality
10. New report reviews science and engineering quality at national security laboratories
11. UCLA scientists receive $2 million grant to improve quality of donor livers for transplant
Post Your Comments:
(Date:4/28/2016)... GOTHENBURG, Sweden , April 28, 2016 ... 1,491.2 M (139.9), up 966% compared with the first quarter of ... Operating profit totaled SEK 589.1 M (loss: 18.8) and the operating ... SEK 7.12 (loss: 0.32) Cash flow from operations was ... , The 2016 revenue guidance is unchanged, SEK 7,000-8,500 M. ...
(Date:4/15/2016)... CHICAGO , April 15, 2016  A ... companies make more accurate underwriting decisions in a ... offering timely, competitively priced and high-value life insurance ... health screenings. With Force Diagnostics, rapid ... and lifestyle data readings (blood pressure, weight, pulse, ...
(Date:3/31/2016)... 2016   LegacyXChange, ... "Company") LegacyXChange is excited to release its ... to be launched online site for trading 100% guaranteed ... will also provide potential shareholders a sense of the ... an industry that is notorious for fraud. The video ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... ... STACS DNA Inc., the sample tracking software company, today announced that Dr. ... STACS DNA as a Field Application Specialist. , “I am thrilled that Dr. ... STACS DNA. “In further expanding our capacity as a scientific integrator, Hays brings a ...
(Date:6/23/2016)... , June 23, 2016 Apellis Pharmaceuticals, ... 1 clinical trials of its complement C3 inhibitor, ... and multiple ascending dose studies designed to assess ... of subcutaneous injection in healthy adult volunteers. ... either as a single dose (ranging from 45 ...
(Date:6/23/2016)... 23, 2016 Andrew ... Published recently in ... journal from touchONCOLOGY, Andrew D Zelenetz , ... cancer care is placing an increasing burden on ... biologic therapies. With the patents on many biologics ...
(Date:6/23/2016)... ... June 23, 2016 , ... ... the Pennsylvania Convention Center and will showcase its product’s latest features from June ... be presenting a scientific poster on Disrupting Clinical Trials in The Cloud during ...
Breaking Biology Technology: