Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α(2)δ-1 Subunit.

Neuron - 26 Apr 2012

Senatore Assunta, Colleoni Simona, Verderio Claudia, Restelli Elena, Morini Raffaella, Condliffe Steven B, Bertani Ilaria, Mantovani Susanna, Canovi Mara, Micotti Edoardo, Forloni Gianluigi, Dolphin Annette C, Matteoli Michela, Gobbi Marco, Chiesa Roberto

Abstract excerpt

How mutant prion protein (PrP) leads to neurological dysfunction in genetic prion diseases is unknown. Tg(PG14) mice synthesize a misfolded mutant PrP which is partially retained in the neuronal endoplasmic reticulum (ER). As these mice age, they develop ataxia and massive degeneration of cerebellar granule neurons (CGNs). Here, we report that motor behavioral deficits in Tg(PG14) mice emerge before...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Biophysics
Share to Electric Stimulation

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Prion Diseases

Share to Prion Proteins

Share to Reaction Time

Share to Rotarod Performance Test

Share to Synaptic Transmission

Share to Synaptosomes

Source

Open in PubMed Open full text

Identifiers

PMID: 22542184
DOI: 10.1016/j.neuron.2012.02.027
PMCID: PMC3339322

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α(2)δ-1 Subunit.PMID 22542184Read ↗

A mutant prion protein sensitizes neurons to glutamate-induced excitotoxicity.Semantic proximity Read ↗

Mutant prion protein enhances NMDA receptor activity, activates PKC, and triggers rapid excitotoxicity in mice.Semantic proximity Read ↗

A transmembrane form of the prion protein is localized in the Golgi apparatus of neurons.Semantic proximity Read ↗

Prion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes.Semantic proximity Read ↗

GFP-tagged prion protein is correctly localized and functionally active in the brains of transgenic mice.Semantic proximity Read ↗

Cellular prion protein acts as mediator of amyloid beta uptake by caveolin‐1 causing cellular dysfunctions in vitro and in vivoSemantic proximity Read ↗

Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia.Semantic proximity Read ↗

Prion protein regulates glutathione metabolism and neural glutamate and cysteine uptake via excitatory amino acid transporter 3.Semantic proximity Read ↗

Parkinson’s-linked LRRK2-G2019S derails AMPAR trafficking, mobility and composition in striatum with cell-type and subunit specificitySemantic proximity Read ↗

Impaired motor activity in a CRISPR SCA5 L253P knock-in mouse is associated with selective β-III-spectrin subcellular redistribution in the cerebellumSemantic proximity Read ↗

Resilience to Endoplasmic Reticulum Stress Mitigates Calcium-Dependent Membrane Hyperexcitability Underlying Late Disease Onset in SCA6Semantic proximity Read ↗

Alexander disease mutant glial fibrillary acidic protein compromises glutamate transport in astrocytes.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.