Molecular link between Parkinson's disease and prion diseases

September 15, 2017, International School of Advanced Studies (SISSA)
Atomic force microscopy of alpha-synuclein amyloid fibrils. Credit: Denis Scaini

Parkinson's disease and prion diseases are very different as regards both origins and course. Nonetheless, a research group of SISSA, headed by Professor Giuseppe Legname, has discovered an unexpected and important link between the two pathologies. According to the study, recently published in the journal Scientific Reports, the pathologies are linked by the complex interaction between two proteins present in nerve cells: the so-called α-synuclein in its aggregated form, and the prion protein PrPC, the molecule responsible, in its altered version, for serious syndromes such as Creutzfeldt-Jakob disease.

The presence of α-synuclein deposits in brain cells is typical of diseases technically called synucleinopathies, which includes Parkinson's disease, characterized by dementia with Lewy bodies and multiple system atrophy. However, the modalities according to which these aggregates form and spread were unknown until now. This researchers discovered that α-synuclein actually makes use of the action of the to spread and deposit in the brain. This seems to favour the formation of these deposits and their spread among .

Additionally, while the activity of the prion protein seems to support the development of synucleopathies, α-synuclein deposits seem to slacken the course of . In fact, this research has proven that α-synuclein fibrils block the deposit of prions in nerve cells, thus preventing their replication. This surprising effect is corroborated by further evidence already found in the pathology—in fact, the course of the disease is slower in patients affected by prion diseases presenting α-synuclein deposits in .

Explore further: Tunneling nanotubes between neurons enable the spread of Parkinson's disease via lysosomes

More information: Suzana Aulić et al, α-Synuclein Amyloids Hijack Prion Protein to Gain Cell Entry, Facilitate Cell-to-Cell Spreading and Block Prion Replication, Scientific Reports (2017). DOI: 10.1038/s41598-017-10236-x

Related Stories

Tunneling nanotubes between neurons enable the spread of Parkinson's disease via lysosomes

August 22, 2016
Scientists from the Institut Pasteur have demonstrated the role of lysosomal vesicles in transporting α-synuclein aggregates, responsible for Parkinson's and other neurodegenerative diseases, between neurons. These proteins ...

Pre-clinical study suggests Parkinson's could start in gut endocrine cells

June 15, 2017
Recent research on Parkinson's disease has focused on the gut-brain connection, examining patients' gut bacteria, and even how severing the vagus nerve connecting the stomach and brain might protect some people from the debilitating ...

Drug discovery: Alzheimer's and Parkinson's spurred by same enzyme

July 3, 2017
Alzheimer's disease and Parkinson's disease are not the same. They affect different regions of the brain and have distinct genetic and environmental risk factors.

Study provides understanding of how nerve cells are damaged by accumulation of abnormal proteins

May 25, 2017
A new study has uncovered a molecular mechanism in the prion protein, a protein responsible for neurodegenerative diseases, which may explain why nerve cells degenerate in these disorders.

Parkinson's disease: New insights into a traveling protein

January 5, 2016
In Parkinson's disease, the protein "alpha-synuclein" aggregates within neurons of patients and appears to propagate across interconnected areas of the brain. How this happens remains largely unknown. It has been proposed ...

Untangling fibril formation and dissociation in Parkinson's disease

November 30, 2016
Prions are misfolded proteins. The complex three-dimensional structure of a prion's progenitor protein becomes altered, somehow causing it to malfunction. Worse, the malformation of these progenitor proteins into prions causes ...

Recommended for you

New transgenic model of Parkinson's illuminates disease biology

October 11, 2018
Parkinson's disease (PD) is a neurodegenerative disorder that presents clinically with abnormal movement and tremors at rest. In the brain, PD is marked by the accumulation of the protein, α-synuclein (αS), into clumps ...

Early Parkinson's patients waiting too long to seek medical evaluation

September 27, 2018
The time between diagnosis and the institution of symptomatic treatment is critical in the effort to find a cure for Parkinson's Disease (PD). A paper published in Nature Partner Journal: Parkinson's Disease notes too many ...

Molecule capable of halting and reverting neurodegeneration caused by Parkinson's disease identified

September 25, 2018
The small SynuClean-D molecule interrupts the formation of the alpha-synuclein amyloid fibres responsible for the onset of Parkinson's disease, and reverts the neurodegeneration caused by the disease. The study, headed by ...

Genomic dark matter activity connects Parkinson's and psychiatric diseases

September 20, 2018
Dopamine neurons are located in the midbrain, but their tendril-like axons can branch far into the higher cortical areas, influencing how we move and how we feel. New genetic evidence has revealed that these specialized cells ...

Gene therapy shown to remove core component of Parkinson's disease

September 14, 2018
An international team led by Rush researcher Jeffrey Kordower, Ph.D., has moved a step closer to developing a treatment to clear brain cells of a protein that is an integral cause of Parkinson's disease. The team published ...

ADHD may increase risk of Parkinson's disease and similar disorders

September 12, 2018
While about 11 percent of children (4-17 years old) nationwide have been diagnosed with attention-deficit hyperactivity disorder (ADHD), the long-term health effects of having ADHD and of common ADHD medications remains understudied. ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.