Tau filaments from amyotrophic lateral sclerosis/parkinsonism-dementia complex adopt the CTE fold.

Autor:	Qi C; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom., Verheijen BM; Leonard Davis School of Gerontology, University of Southern California, Los Angeles, CA 90089., Kokubo Y; Graduate School of Regional Innovation Studies, Mie University, Tsu 514-8507, Japan., Shi Y; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom., Tetter S; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom., Murzin AG; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom., Nakahara A; Department of Pathology, Brain Research Institute, Niigata University, Niigata 951-8585, Japan., Morimoto S; Department of Oncologic Pathology, Graduate School of Medicine, Mie University, Tsu 514-8507, Japan., Vermulst M; Leonard Davis School of Gerontology, University of Southern California, Los Angeles, CA 90089., Sasaki R; Department of Nursing, Suzuka University of Medical Science, Suzuka 513-8670, Japan., Aronica E; Department of Neuropathology, Amsterdam University Medical Centers (UMC), University of Amsterdam, Amsterdam Neuroscience, Amsterdam 1105 AZ, The Netherlands., Hirokawa Y; Department of Oncologic Pathology, Graduate School of Medicine, Mie University, Tsu 514-8507, Japan., Oyanagi K; Department of Brain Disease Research, Shinshu University School of Medicine, Matsumoto 390-8621, Japan., Kakita A; Department of Pathology, Brain Research Institute, Niigata University, Niigata 951-8585, Japan., Ryskeldi-Falcon B; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom., Yoshida M; Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute 480-1195, Japan., Hasegawa M; Department of Brain and Neuroscience, Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, Japan., Scheres SHW; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom., Goedert M; Medical Research Council, Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom.
Jazyk:	angličtina
Zdroj:	Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2023 Dec 19; Vol. 120 (51), pp. e2306767120. Date of Electronic Publication: 2023 Dec 15.
DOI:	10.1073/pnas.2306767120
Abstrakt:	The amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) of the island of Guam and the Kii peninsula of Japan is a fatal neurodegenerative disease of unknown cause that is characterized by the presence of abundant filamentous tau inclusions in brains and spinal cords. Here, we used electron cryo-microscopy to determine the structures of tau filaments from the cerebral cortex of three cases of ALS/PDC from Guam and eight cases from Kii, as well as from the spinal cord of two of the Guam cases. Tau filaments had the chronic traumatic encephalopathy (CTE) fold, with variable amounts of Type I and Type II filaments. Paired helical tau filaments were also found in three Kii cases and tau filaments with the corticobasal degeneration fold in one Kii case. We identified a new Type III CTE tau filament, where protofilaments pack against each other in an antiparallel fashion. ALS/PDC is the third known tauopathy with CTE-type filaments and abundant tau inclusions in cortical layers II/III, the others being CTE and subacute sclerosing panencephalitis. Because these tauopathies are believed to have environmental causes, our findings support the hypothesis that ALS/PDC is caused by exogenous factors. Competing Interests: Competing interests statement:The authors declare no competing interest.
Databáze:	MEDLINE
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