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Unfolded protein response activation in C9orf72 frontotemporal dementia is associated with dipeptide pathology and granulovacuolar degeneration in granule cells.
Gami-Patel P, van Dijken I, Meeter LH, Melhem S, Morrema THJ, Scheper W, van Swieten JC, Rozemuller AJM, Dijkstra AA, Hoozemans JJM. Gami-Patel P, et al. Among authors: van dijken i. Brain Pathol. 2021 Jan;31(1):163-173. doi: 10.1111/bpa.12894. Epub 2020 Sep 16. Brain Pathol. 2021. PMID: 32865835 Free PMC article.
Von Economo neurons are part of a larger neuronal population that are selectively vulnerable in C9orf72 frontotemporal dementia.
Gami-Patel P, van Dijken I, van Swieten JC, Pijnenburg YAL; Netherlands Brain Bank; Rozemuller AJM, Hoozemans JJM, Dijkstra AA. Gami-Patel P, et al. Among authors: van dijken i. Neuropathol Appl Neurobiol. 2019 Dec;45(7):671-680. doi: 10.1111/nan.12558. Epub 2019 Jun 10. Neuropathol Appl Neurobiol. 2019. PMID: 31066065 Free PMC article.