Protein transmission in neurodegenerative disease.

Protein transmission in neurodegenerative disease.

Publication date: Mar 23, 2020

Most neurodegenerative diseases are characterized by the intracellular or extracellular aggregation of misfolded proteins such as amyloid-? and tau in Alzheimer disease, ?-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.

Peng, C., Trojanowski, J.Q., and Lee, V.M. Protein transmission in neurodegenerative disease. 24442. 2020 Nat Rev Neurol.

Concepts Keywords
Alzheimer Branches of biology
Amyloid Organ systems
Amyotrophic Lateral Sclerosis Neurological disorders
Conformation Neuroscience
DNA Neurodegeneration
Extracellular Senescence
Intracellular Amyloidosis
Neurodegenerative Disease Prion
Neurodegenerative Diseases Benjamin Wolozin
Parkinson Disease DNA binding

Semantics

Type Source Name
disease MESH neurodegenerative disease
pathway REACTOME Neurodegenerative Diseases
disease MESH Alzheimer disease
pathway KEGG Alzheimer disease
disease MESH Parkinson disease
pathway KEGG Parkinson disease
disease MESH amyotrophic lateral sclerosis

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