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Robust α-synuclein pathology in select brainstem neuronal populations is a potential instigator of multiple system atrophy
Ethan W Hass 1 2, Zachary A Sorrentino 1 2, Grace M Lloyd 1 2, Nikolaus R McFarland 2 3 4, Stefan Prokop 2 3 5, Benoit I Giasson 6 7 8
Abstract:
...Although (glial cytoplasmic inclusions, GCIs) are the hallmark of MSA, using a series of new monoclonal antibodies targeting the carboxy-terminal region of αSyn, we demonstrate that neuronal αSyn pathology in MSA patient brains is remarkably abundant in the pontine nuclei and medullary inferior olivary nucleus. This neuronal αSyn pathology has distinct histological properties compared to GCIs, which allows it to remain concealed to many routine detection methods associated with altered biochemical properties of the carboxy-terminal domain of αSyn. We propose that these previously underappreciated sources of aberrant αSyn could serve as a pool of αSyn prion seeds that can initiate and continue to drive the pathogenesis of MSA.
