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

Pathogenic Ubqln2 gains toxic properties to induce neuron death.

Acta neuropathologica - 1 Mar 2015

Wu Qinxue, Liu Mujun, Huang Cao, Liu Xionghao, Huang Bo, Li Niansheng, Zhou Hongxia, Xia Xu-Gang

Abstract excerpt

Mutations in ubiquilin 2 (Ubqln2) is linked to amyotrophic lateral sclerosis and frontotemporal lobar degeneration. A foremost question regarding Ubqln2 pathogenesis is whether pathogenically mutated Ubqln2 causes neuron death via a gain or loss of functions. To better understand Ubqln2 pathobiology, we created Ubqln2 transgenic and knockout rats and compared phenotypic expression in these novel rat models....

Read the complete abstract on PubMed

Topics

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

Share to Cell Death
Share to Gene Knockout Techniques
Share to Immunoprecipitation

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Microscopy, Electron, Transmission

Share to Nerve Degeneration

Share to Rats, Sprague-Dawley

Share to Rats, Transgenic

Share to Ubiquitins

Source

Open in PubMed Open full text

Identifiers

PMID: 25388785
DOI: 10.1007/s00401-014-1367-y
PMCID: PMC4777328

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

Pathogenic Ubqln2 gains toxic properties to induce neuron death.PMID 25388785Read ↗

Increased Ubqln2 expression causes neuron death in transgenic rats.Semantic proximity Read ↗

UBQLN2 in neurodegenerative disease: mechanistic insights and emerging therapeutic potential.Semantic proximity Read ↗

Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in ratsSemantic proximity Read ↗

Dendritic spinopathy in transgenic mice expressing ALS/dementia-linked mutant UBQLN2.Semantic proximity Read ↗

Modeling UBQLN2-mediated neurodegenerative disease in mice: Shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity.Semantic proximity Read ↗

Viral expression of ALS-linked ubiquilin-2 mutants causes inclusion pathology and behavioral deficits in miceSemantic proximity Read ↗

Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementiaSemantic proximity Read ↗

Disturbance of proteasomal and autophagic protein degradation pathways by amyotrophic lateral sclerosis-linked mutations in ubiquilin 2.Semantic proximity Read ↗

Chronic Oxidative Stress and Stress Granule Formation in UBQLN2 ALS Neurons: Insights into Neuronal Degeneration and Potential Therapeutic TargetsSemantic proximity Read ↗

Loss ofALS2Function Is Insufficient to Trigger Motor Neuron Degeneration in Knock-Out Mice But Predisposes Neurons to Oxidative StressSemantic proximity Read ↗

UBQLN2 links proteotoxicity with lipid metabolism in neurodegeneration.Semantic proximity Read ↗

Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stressSemantic proximity Read ↗

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