Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism
University of California, San Francisco · Institute for Neurodegenerative Disorders · +8 more institutions
Abstract
Prions are proteins that adopt alternative conformations that become self-propagating; the PrP(Sc) prion causes the rare human disorder Creutzfeldt-Jakob disease (CJD). We report here that multiple system atrophy (MSA) is caused by a different human prion composed of the α-synuclein protein. MSA is a slowly evolving disorder characterized by progressive loss of autonomic nervous system function and often signs of parkinsonism; the neuropathological hallmark of MSA is glial cytoplasmic inclusions consisting of filaments of α-synuclein. To determine whether human α-synuclein forms prions, we examined 14 human brain homogenates for transmission to cultured human embryonic kidney (HEK) cells expressing…
Citation impact
- FWCI
- 50.70
- Percentile
- 100%
- References
- 65
Authors
17- SBStanley B. PrusinerCorresponding
University of California, San Francisco, Institute for Neurodegenerative Disorders
- ALAmanda L. Woerman
University of California, San Francisco, Institute for Neurodegenerative Disorders
- DADaniel A. Mordes
Harvard University, Massachusetts General Hospital
- JCJoel C. Watts
University of California, San Francisco, Institute for Neurodegenerative Disorders
- RRRyan Rampersaud
University of California, San Francisco, Institute for Neurodegenerative Disorders
Topics & keywords
- Parkinsonism
- Atrophy
- Neuroscience
- Medicine
- Pathology
- Biology
- Disease
- Good health and well-being
Funding
- SFSherman Fairchild Foundation
- NRNeuroscience Research Australia
- PUParkinson's UKAwards: 948776; SC037554, 948776
- ICImperial College London
- UOUniversity of New South Wales
- NINational Institutes of HealthAwards: AG031220, AG010770, AG002132, AG005134, AG021601
- MRMedical Research Council
- NHNational Health and Medical Research CouncilAward: 1079679