“If waste is not removed it will build up over time and become toxic, causing cells to go wrong and eventually die.”
Faults in this system may play an important role in neurodegenerative diseases such as Parkinson’s and Alzheimer’s because they are caused by the death of neurons – the network through which we transfer information in our brain.
Harmful moleculesUsing gene targeting in mice, the researchers have discovered that a faulty UPS in neurons leads to damaged mitochondria that produce less energy. Damages mitochondria are also known to produce harmful molecules that injure the cell – oxidative stress – so it is vital that the brain is able to keep mending, removing and replacing them.
The study also found that when the UPS was faulty, the damaged mitochondria were not removed from neurons in the normal way by the process of autophagy, the disposal system that breaks down larger parts in the cell like mitochondria.
The research was conducted in collaboration with experts at Nottingham Trent University, the University of Dundee, Nottingham University Hospitals NHS Trust and Great Ormond Street Hospital for Children NHS Trust in the UK and the University of Tripoli in Libya.
A copy of the research paper, Continued 26S proteasome dysfunction in mouse braincortical neurons impairs autophagy and the Keap1-Nrf2 oxidative defence pathway, is available online.