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Prions induce an early Arc response and a subsequent reduction in mGluR5 in the hippocampus.

Neurobiology of disease · 2022 · Vol. 172 · pp. 105834

Abstract

Synapse dysfunction and loss are central features of neurodegenerative diseases, caused in part by the accumulation of protein oligomers. Amyloid-β, tau, prion, and α-synuclein oligomers bind to the cellular prion protein (PrP<sup>C),</sup> resulting in the activation of macromolecular complexes and signaling at the post-synapse, yet the early signaling events are unclear. Here we sought to determine the early transcript and protein alterations in the hippocampus during the pre-clinical stages of prion disease. We used a transcriptomic approach focused on the early-stage, prion-infected hippocampus of male wild-type mice, and identify immediate early genes, including the synaptic activity response gene, Arc/Arg3.1, as significantly upregulated. In a longitudinal study of male, prion-infected mice, Arc/Arg-3.1 protein was increased early (40% of the incubation period), and by mid-disease (pre-clinical), phosphorylated AMPA receptors (pGluA1-S845) were increased and metabotropic glutamate receptors (mGluR5 dimers) were markedly reduced in the hippocampus. Notably, sporadic Creutzfeldt-Jakob disease (sCJD) post-mortem cortical samples also showed low levels of mGluR5 dimers. Together, these findings suggest that prions trigger an early Arc response, followed by an increase in phosphorylated GluA1 and a reduction in mGluR5 receptors.

Publication Types

["Journal Article", "Research Support, Non-U.S. Gov't", "Research Support, N.I.H., Extramural"]

Keywords

MeSH Terms

["Amyloid beta-Peptides", "Animals", "Creutzfeldt-Jakob Syndrome", "Hippocampus", "Longitudinal Studies", "Male", "Mice", "Prions"]

Funding

R01 NS069566 NINDS NIH HHS (United States)
P30 NS047101 NINDS NIH HHS (United States)
F31 NS103588 NINDS NIH HHS (United States)
RF1 NS121992 NINDS NIH HHS (United States)
R01 NS076896 NINDS NIH HHS (United States)
P30 AG062429 NIA NIH HHS (United States)
R56 AG055619 NIA NIH HHS (United States)
R01 AG031189 NIA NIH HHS (United States)
S10 OD023527 NIH HHS (United States)
R01 NS121992 NINDS NIH HHS (United States)
R01 AG062562 NIA NIH HHS (United States)
R37 NS076896 NINDS NIH HHS (United States)

Linked Datasets (1)

GSE207251 GSE via ncbi_elink
GEO

Prions induce an early Arc response in the hippocampus of prion-infected mice

Mus musculus
6 data files
FileTypeSize
2712_1_S25_L003_R1_001.fastq.gz RNA-Seq 1.6 GB
2712_2_S26_L003_R1_001.fastq.gz RNA-Seq 2.2 GB
2712_3_S27_L003_R1_001.fastq.gz RNA-Seq 1.6 GB
2713_2_S28_L003_R1_001.fastq.gz RNA-Seq 2.0 GB
2713_3_S29_L003_R1_001.fastq.gz RNA-Seq 1.9 GB
2713_4_S30_L003_R1_001.fastq.gz RNA-Seq 1.5 GB

Potentially Related Datasets (1)

These accessions were text-mined from the PMC full text. They may be referenced for comparison, cited from other studies, or otherwise mentioned without being primary data for this paper.

Prions induce an early Arc response in the hippocampus of prion-infected mice

Analysis Pipelines (1)

RNA-seq geo_data_processing GSE207251