By discovering a mechanism by which mitochondria — tiny structures inside cells often described as «power plants» — signal that they are damaged and need to be eliminated, the Pitt team has opened the door to potential research into cures for disorders such as Parkinson's disease that are believed to be caused by
dysfunctional mitochondria in neurons.
Neuroscientist Patrik Verstreken of VIB (Flanders Institute for Biotechnology) and KU Leuven has shown for the first time that
dysfunctional mitochondria in brain cells can lead to learning disabilities.
The study, published Feb. 5 in Nature Neuroscience, showed that the patients» nerve cells — converted directly from patients» skin cells — exhibited «symptoms» of the disorder, including DNA damage,
dysfunctional mitochondria and cell death.
Because mitochondria are so vital to a cell's normal functions, damaged and
dysfunctional mitochondria have been implicated in a wide array of diseases and disorders, such as diabetes and schizophrenia.
These dysfunctional mitochondria led to increased oxidized dopamine levels, creating a vicious cycle.
Mitochondria can be damaged as people age and cells experience various forms of stress, and it is the job of PINK1 to help trigger a process called mitophagy to remove
those dysfunctional mitochondria.
Deprived of energy,
the dysfunctional mitochondria aggravate the inflammation that causes muscle loss.
In a normal mitochondrion (left) these folds fill the interior, but these folds are lost in damaged or
dysfunctional mitochondria (right).
Scientists at the NIH's National Institute of Neurological Disorders and Stroke (NINDS) showed that a protein called PINK1 that is implicated in Parkinson's disease is critical for helping cells get rid of
dysfunctional mitochondria.
The Leuven - based VIB researchers Dominik Haddad, Vanessa Morais and Patrik Verstreken have unraveled the mechanism by which brain cells trigger the destruction of
dysfunctional mitochondria.
Earlier research has shown that Parkinson's disease is often paired with
dysfunctional mitochondria.
Moreover,
dysfunctional mitochondria are not efficiently discarded from the cell, which complicates the operation of other healthy mitochondria and leads to insufficient energy production in the cell.
In 2016, the researchers reported that hypoxia, or oxygen deficiency, actually improves the health of mice genetically engineered to have
dysfunctional mitochondria, tiny power - producing organelles.
Now, scientists at the Salk Institute have uncovered an unexpected way in which cells trigger this critical response to threats, offering insight into disorders such as mitochondrial disease, cancer, diabetes and neurodegenerative disease — particularly Parkinson's disease, which is linked to
dysfunctional mitochondria.
A defective endosomal — lysosomal autophagy pathway (due to either a primary defect in this pathway or
dysfunctional mitochondria) could be the common denominator in various forms of ALS.
Fasting also stimulates autophagy and mitophagy, the process of culling the old,
dysfunctional mitochondria.
Dysfunctional mitochondria can no longer produce enough energy and «starving» cells may degenerate and die.
This leads to a decline in energy levels in the brain, and
these dysfunctional mitochondria release larger amounts of free radicals that can damage the brain cells.