UCSF continues to say that MSA is a prion disorder

Sigh….  Some UCSF folks continue to say that multiple system atrophy is a prion disease, which means that it’s infectious if brain tissue is touched.  This has really gummed up the works for brain donation in two places in the US.

The abstract is copied below.



Cold Spring Harbor Perspectives in Medicine. 2017 Feb 17.

α-Synuclein: Multiple System Atrophy Prions.
Woerman AL, Watts JC, Aoyagi A, Giles K, Middleton LT, Prusiner SB.

Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disease arising from the misfolding and accumulation of the protein α-synuclein in oligodendrocytes, where it forms glial cytoplasmic inclusions (GCIs). Several years of studying synthetic α-synuclein fibrils has provided critical insight into the ability of α-synuclein to template endogenous protein misfolding, giving rise to fibrillar structures capable of propagating from cell to cell. However, more recent studies with MSA-derived α-synuclein aggregates have shown that they have a similar ability to undergo template-directed propagation, like PrP prions. Almost 20 years after α-synuclein was discovered as the primary component of GCIs, α-synuclein aggregates isolated from MSA patient samples were shown to infect cultured mammalian cells and also to transmit neurological disease to transgenic mice. These findings argue that α-synuclein becomes a prion in MSA patients. In this review, we discuss the in vitro and in vivo data supporting the recent classification of MSA as a prion disease.

Copyright © 2017 Cold Spring Harbor Laboratory Press; all rights reserved.

PubMed ID#: 28213437 (see pubmed.gov for the abstract only)