Alpha-synuclein
- OMIM: 163890
- UniProt: P37840
α -synuclein ( α - synuclein also ) is a small, soluble protein in the brains of vertebrates that regulates dopamine release among others. It is able to form membrane channels, and is therefore a transport protein. Mutations in SNCA gene are responsible for the so-called synucleinopathies such as hereditary forms 1 and 4, of Parkinson's disease and Lewy body dementia.
Function
As the name implies, SNCA initially identified as synaptic and nuclear protein. Despite intensive studies, the exact role of SNCA is not yet clearly defined, there is evidence that SNCA in the maintenance of synaptic vesicle pools plays a role and modulates dopamine release, but SNCA knockout mice have no obvious phenotype.
Pathology
It has been identified in the human brain as a precursor of the non- β -amyloid protein. Scientists from the U.S. National Human Genome Research Institute ( NHGRI ) at the National Institutes of Health ( NIH) in Bethesda, Maryland in 1997 found that diseases such as the Parkinson's disease is involved in several pathogenic processes in neurodegenerative diseases. Thus, a positive immune reaction for antibodies against α -synuclein was found in the typical for the latter Lewy bodies. This protein is toxic to certain nerve cells, but especially on dopaminergic neurons of the substantia nigra attributed, in which it is considered in the form of protofibrils as a contributory cause oxidative stress and resulting neuronal cell death. There is discussed a meaning of α - Synucleine in the pathogenesis of prion disease, or Alzheimer's disease.
The group of neurodegenerative disorders in which there is a pathological accumulation of α -synuclein in the central nervous system is known as synucleinopathies.
The Viennese company AFFiRiS leads to develop with the aim of a vaccine against Parkinson's disease since June 5, 2012, a clinical trial with the anti- α -synuclein drug PD01A by. Through vaccination, the immune system should be stimulated to produce antibodies, which are directed against α -synuclein.