Prusiner SB, Woerman AL, Mordes DA, Watts JC, Rampersaud R, Berry DB, Patel S, Oehler A, Lowe JK, Kravitz SN, Geschwind DH, Glidden DV, Halliday GM, Middleton LT, Gentleman SM, Grinberg LT, Giles K. Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism. Proc Natl Acad Sci U S A. 2015 Sep 22;112(38):E5308-17. Epub 2015 Aug 31 PubMed. Correction.

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  1. This paper expands on previous work in which the authors tested two MSA patient samples (Watts et a., 2013). In the current study, the authors tested samples from 12 more MSA patients and 6 PD patients and found that only the MSA brain homogenates transmit the disease to the α-synuclein transgenic mice. These are interesting data. The paper shows that the transmission of synucleinopathy is context-dependent. MSA brain extracts are certainly more potent than those from PD patients. This doesn't necessarily prove the strain-disease relationship since we don't even know whether the phenotypes we see in the inoculated mice are relevant to MSA or to any other human diseases. One caveat is that the anti-phospho-α-Syn antibody may have non-specific interactions with other proteins. Pathological examination with antibody to total alpha-synuclein might help validate the results. The million-dollar question is whether the pathology resulted from the seeded aggregation of α-synuclein.

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  1. α-Synuclein from Multiple System Atrophy Acts Like Prion in Mice