Pink1 observed in action, a protein that helps to replace the damaged mitochondria and which is changed in some forms of Parkinson’s.
A key protein to get rid of the damaged mitochondria, and which in a changed form is connected directly to Parkinson’s disease, has been observed for the first time in action: a conquest that could help the development of new drugs against this pathology.
In a study published on Sciencethe scientists of the Wehi Parkinson’s Disease Research Center in Australia have finally determined the structure of the proteincall Pink1. By understanding its attachment mechanisms to mitochondria and activation.
Central affected. Even if Pink1 has been known for 20 years, nobody has studied the structure in detail so far, nor observed the way in which it adheres to the surface of the damaged mitochondria. Mitochondria produces cellular energy and cells that need a lot of energy, such as brain ones, can contain hundreds or thousands. It follows that, if the mitochondria are damaged, brain cells are particularly vulnerable to damage.
The pathology. Parkinson’s is a neurodegenerative disease in which they are affected above all The neurons of the mesencefaloinvolved in the body’s motor functions. The death of neurons leads to manifest symptoms such as tremors, rigidity, slowdown in movements, disturbances of balance, language and swallowing, as well as other dozens of secondary disorders.
Dangerous accumulations. The Pink1 protein, produced by Gene Park6, has the task of identify the damaged mitochondria and to report them as “to be removed”.
In a healthy person, the protein accumulates on the membranes of the mitochondria and, through another signal protein called ubiquitinhe says to the body that damaged mitochondria must be removed. When Pink1 instead changed, the damaged mitochondria accumulate inside the cells. A mutation in the gene that codes the Pink1 protein would seem to the base of many cases of youth parkinson, a form of early debut parkinson and on a genetic basis that affects people with less than 45 years.
A big step forward. The authors of the study discovered that Pink1 works in 4 phases Fundamental, the first two of which had never been studied in detail so far. Initially he warns the mitochondrial damage; Then he attacks the damaged mitochondria; At this point, it reports to ubiquitin, which in turn connects to a protein called Parkin so that the damaged mitochondrium can be recycled.
«This is the first time we see the human Pink1 anchored to the surface of the damaged mitochondria and we discovered a significant range of proteins that act as an docking site.
We also saw, for the first time, how the mutations present in people with Parkinson’s disease influence human Pink1, “says Sylvie Callegari, senior author of the study.
Having clarified better and activation of the protein will facilitate the development of targeted pharmacological therapies, a step that has not been possible so far.
