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

Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.

Molecular neurodegeneration - 28 Jul 2016

Alves Sandro, Marais Thibaut, Biferi Maria-Grazia, Furling Denis, Marinello Martina, El Hachimi Khalid, Cartier Nathalie, Ruberg Merle, Stevanin Giovanni, Brice Alexis, Barkats Martine, Sittler Annie

Abstract excerpt

BACKGROUND: We used lentiviral vectors (LVs) to generate a new SCA7 animal model overexpressing a truncated mutant ataxin-7 (MUT ATXN7) fragment in the mouse cerebellum, in order to characterize the specific neuropathological and behavioral consequences of the genetic defect in this brain structure. RESULTS: LV-mediated overexpression of MUT ATXN7 into the cerebellum of C57/BL6 adult mice induced...

Read the complete abstract on PubMed

Topics

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

Share to Ataxin-7
Share to Disease Models, Animal

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to DNA-Binding Proteins

Share to Lentivirus

Share to Mice, Inbred C57BL

Share to Neurons

Share to RNA-Binding Proteins

Share to Spinocerebellar Ataxias

Source

Open in PubMed Open full text

Identifiers

PMID: 27465358
DOI: 10.1186/s13024-016-0123-2
PMCID: PMC4964261

Genes

ATXN7
FUS
MBNL1
MBNL2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.PMID 27465358Read ↗

Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.Semantic proximity Read ↗

Disrupting ATXN1 Nuclear Localization in a Knock-in SCA1 Mouse Model Improves a Spectrum of SCA1-Like Phenotypes and their Brain Region Associated Transcriptomic ProfilesSemantic proximity Read ↗

AAV-Mediated CAG-Targeting Selectively Reduces Polyglutamine-Expanded Protein and Attenuates Disease Phenotypes in a Spinocerebellar Ataxia Mouse Model.Semantic proximity Read ↗

SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types.Semantic proximity Read ↗

Decreasing mutant ATXN1 nuclear localization improves a spectrum of SCA1-like phenotypes and brain region transcriptomic profiles.Semantic proximity Read ↗

Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice.Semantic proximity Read ↗

Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration.Semantic proximity Read ↗

Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration.Semantic proximity Read ↗

AAV-based delivery of RNAi targeting Ataxin-2 improves survival, strength, and pathology in mouse models of rapidly and slowly progressive sporadic ALSSemantic proximity Read ↗

A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes.Semantic proximity Read ↗

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.Semantic proximity Read ↗

Transgenic mice with SCA10 pentanucleotide repeats show motor phenotype and susceptibility to seizure: a toxic RNA gain-of-function model.Semantic proximity Read ↗

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