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

Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: conclusions from double-transgenic mouse models.

Neurobiology of disease - 1 Apr 2010

Hübener Jeannette, Riess Olaf

Abstract excerpt

A crucial question in polyQ-induced neurodegeneration is the influence of wild type protein on the formation of aggregates and toxicity. Recently it was shown that non-expanded ataxin-3 protein mitigated neurodegeneration in a Drosophila and mouse model of SCA3. We now explored the effects of overexpressing non-expanded ataxin-3 with 15Q in a SCA3 transgenic mouse model with 70 polyglutamine repeats. These...

Read the complete abstract on PubMed

Topics

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

Share to Ataxin-3
Share to Genetic Therapy
Share to Inclusion Bodies

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Machado-Joseph Disease

Share to Nerve Degeneration

Share to Nuclear Export Signals

Share to Survival Rate

Share to Trinucleotide Repeat Expansion

Source

Identifiers

PMID: 20079840
DOI: 10.1016/j.nbd.2010.01.005

Genes

NES

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

Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: conclusions from double-transgenic mouse models.PMID 20079840Read ↗

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.Semantic proximity Read ↗

Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models.Semantic proximity Read ↗

A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes.Semantic proximity Read ↗

T1-11 and JMF1907 ameliorate polyglutamine-expanded ataxin-3-induced neurodegeneration, transcriptional dysregulation and ataxic symptom in the SCA3 transgenic mouseSemantic proximity Read ↗

Inhibition of transglutaminase exacerbates polyglutamine-induced neurotoxicity by increasing the aggregation of mutant ataxin-3 in an SCA3 Drosophila model.Semantic proximity Read ↗

A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats.Semantic proximity Read ↗

N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation.Semantic proximity Read ↗

A novel Ataxin-3 knock-in mouse model mimics the human SCA3 disease phenotype including neuropathological, behavioral, and transcriptional abnormalitiesSemantic proximity Read ↗

Large-Scale Screen for Modifiers of Ataxin-3-Derived Polyglutamine-Induced Toxicity in DrosophilaSemantic proximity Read ↗

Mouse ataxin-3 functional knock-out model.Semantic proximity Read ↗

Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: removal of the CAG containing exon.Semantic proximity Read ↗

A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in OligodendrocytesSemantic proximity Read ↗

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