Pathological stress granules in Alzheimer’s disease

Abstract A feature of neurodegenerative disease is the accumulation of insoluble protein aggregates in the brain. In some conditions, including Amyotrophic Lateral Sclerosis and Frontotemporal lobar degeneration, the primary aggregating entities are RNA binding proteins. Through regulated prion-like...

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Bibliographic Details
Published in:	Brain research Vol. 1584; pp. 52 - 58
Main Authors:	Ash, Peter E.A, Vanderweyde, Tara E, Youmans, Katherine L, Apicco, Daniel J, Wolozin, Benjamin
Format:	Journal Article Conference Proceeding
Language:	English
Published:	Amsterdam Elsevier B.V 10-10-2014 Elsevier
Subjects:	Adult and adolescent clinical studies Alzheimer Disease - metabolism Alzheimer Disease - pathology Alzheimer’s disease Animals Biological and medical sciences Brain - metabolism Brain - pathology Carrier Proteins - metabolism Cytoplasmic Granules - metabolism Cytoplasmic Granules - pathology Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases DNA Helicases DNA-Binding Proteins - metabolism G3BP1 Humans Medical sciences Mice Neurology Organic mental disorders. Neuropsychology Pathological stress granules Poly(A)-Binding Proteins - metabolism Poly-ADP-Ribose Binding Proteins Protein Aggregation, Pathological - metabolism Psychology. Psychoanalysis. Psychiatry Psychopathology. Psychiatry RNA Helicases RNA Recognition Motif Proteins RNA-Binding Proteins - metabolism Signal Transduction Stress, Physiological T-Cell Intracellular Antigen-1 Tau TIA-1 Tristetraprolin - metabolism TIA-1 Tau Alzheimer’s disease Pathological stress granules G3BP1 Nervous system diseases Tau protein Alzheimer disease Central nervous system disease Degenerative disease Alzheimer's disease Stress Cerebral disorder
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Summary:	Abstract A feature of neurodegenerative disease is the accumulation of insoluble protein aggregates in the brain. In some conditions, including Amyotrophic Lateral Sclerosis and Frontotemporal lobar degeneration, the primary aggregating entities are RNA binding proteins. Through regulated prion-like assembly, RNA binding proteins serve many functions in RNA metabolism that are essential for the healthy maintenance of cells of the central nervous system. Those RNA binding proteins that are the core nucleating factors of stress granules (SGs), including TIA-1, TIAR, TTP and G3BP1, are also found in the pathological lesions of other neurological conditions, such as Alzheimer’s disease, where the hallmark aggregating protein is not an RNA binding protein. This discovery suggests that the regulated cellular pathway, which utilizes assembly of RNA binding proteins to package and silence mRNAs during stress, may be integral in the aberrant pathological protein aggregation that occurs in numerous neurodegenerative conditions. This article is part of a Special Issue entitled RNA Metabolism 2013.
ISSN:	0006-8993 1872-6240
DOI:	10.1016/j.brainres.2014.05.052