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Protein transmission in neurodegenerative disease.
Nat Rev Neurol. 2020 04; 16(4):199-212.NR

Abstract

Most neurodegenerative diseases are characterized by the intracellular or extracellular aggregation of misfolded proteins such as amyloid-β and tau in Alzheimer disease, α-synuclein in Parkinson disease, and TAR DNA-binding protein 43 in amyotrophic lateral sclerosis. Accumulating evidence from both human studies and disease models indicates that intercellular transmission and the subsequent templated amplification of these misfolded proteins are involved in the onset and progression of various neurodegenerative diseases. The misfolded proteins that are transferred between cells are referred to as 'pathological seeds'. Recent studies have made exciting progress in identifying the characteristics of different pathological seeds, particularly those isolated from diseased brains. Advances have also been made in our understanding of the molecular mechanisms that regulate the transmission process, and the influence of the host cell on the conformation and properties of pathological seeds. The aim of this Review is to summarize our current knowledge of the cell-to-cell transmission of pathological proteins and to identify key questions for future investigation.

Authors+Show Affiliations

Department of Neurology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA.The Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.The Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. vmylee@upenn.edu.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

32203399

Citation

Peng, Chao, et al. "Protein Transmission in Neurodegenerative Disease." Nature Reviews. Neurology, vol. 16, no. 4, 2020, pp. 199-212.
Peng C, Trojanowski JQ, Lee VM. Protein transmission in neurodegenerative disease. Nat Rev Neurol. 2020;16(4):199-212.
Peng, C., Trojanowski, J. Q., & Lee, V. M. (2020). Protein transmission in neurodegenerative disease. Nature Reviews. Neurology, 16(4), 199-212. https://doi.org/10.1038/s41582-020-0333-7
Peng C, Trojanowski JQ, Lee VM. Protein Transmission in Neurodegenerative Disease. Nat Rev Neurol. 2020;16(4):199-212. PubMed PMID: 32203399.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Protein transmission in neurodegenerative disease. AU - Peng,Chao, AU - Trojanowski,John Q, AU - Lee,Virginia M-Y, Y1 - 2020/03/23/ PY - 2020/02/18/accepted PY - 2020/3/24/pubmed PY - 2020/4/29/medline PY - 2020/3/24/entrez SP - 199 EP - 212 JF - Nature reviews. Neurology JO - Nat Rev Neurol VL - 16 IS - 4 N2 - Most neurodegenerative diseases are characterized by the intracellular or extracellular aggregation of misfolded proteins such as amyloid-β and tau in Alzheimer disease, α-synuclein in Parkinson disease, and TAR DNA-binding protein 43 in amyotrophic lateral sclerosis. Accumulating evidence from both human studies and disease models indicates that intercellular transmission and the subsequent templated amplification of these misfolded proteins are involved in the onset and progression of various neurodegenerative diseases. The misfolded proteins that are transferred between cells are referred to as 'pathological seeds'. Recent studies have made exciting progress in identifying the characteristics of different pathological seeds, particularly those isolated from diseased brains. Advances have also been made in our understanding of the molecular mechanisms that regulate the transmission process, and the influence of the host cell on the conformation and properties of pathological seeds. The aim of this Review is to summarize our current knowledge of the cell-to-cell transmission of pathological proteins and to identify key questions for future investigation. SN - 1759-4766 UR - https://www.unboundmedicine.com/medline/citation/32203399/Protein_transmission_in_neurodegenerative_disease_ L2 - http://dx.doi.org/10.1038/s41582-020-0333-7 DB - PRIME DP - Unbound Medicine ER -