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

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

The Journal of neuroscience : the official journal of the Society for Neuroscience - 11 Jul 2007

Bichelmeier Ulrike, Schmidt Thorsten, Hübener Jeannette, Boy Jana, Rüttiger Lukas, Häbig Karina, Poths Sven, Bonin Michael, Knipper Marlies, Schmidt Werner J, Wilbertz Johannes, Wolburg Hartwig, Laccone Franco, Riess Olaf

Abstract excerpt

Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly inherited neurodegenerative disorder caused by the expansion of a CAG repeat in the MJD1 gene resulting in an expanded polyglutamine repeat in the ataxin-3 protein. To study the course of the disease, we generated transgenic mice for SCA3 using full-length ataxin-3 constructs containing 15, 70, or 148 CAG repeats, respectively. Control mice (15 CAGs)...

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 Exploratory Behavior

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Inclusion Bodies

Share to Machado-Joseph Disease

Share to Nerve Degeneration

Share to Staining and Labeling

Share to Tremor

Share to Trinucleotide Repeats

Source

Open in PubMed Open full text

Identifiers

PMID: 17626202
DOI: 10.1523/JNEUROSCI.4540-06.2007
PMCID: PMC6672614

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

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.PMID 17626202Read ↗

An Isoform of Ataxin‐3 Accumulates in the Nucleus of Neuronal Cells in Affected Brain Regions of SCA3 PatientsSemantic 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 ↗

Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology.Semantic proximity Read ↗

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

Autophagy in Spinocerebellar Ataxia Type 3: From Pathogenesis to TherapeuticsSemantic proximity Read ↗

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

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.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 ↗

Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.Semantic proximity Read ↗

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

Machado–Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapySemantic proximity Read ↗

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