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

Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.

Cell death & disease - 16 Aug 2012

Yin H Z, Nalbandian A, Hsu C-I, Li S, Llewellyn K J, Mozaffar T, Kimonis V E, Weiss J H

Abstract excerpt

Pathological features of amyotrophic lateral sclerosis (ALS) include, in addition to selective motor neuron (MN) degeneration, the occurrence of protein aggregates, mitochondrial dysfunction and astrogliosis. SOD1 mutations cause rare familial forms of ALS and have provided the most widely studied animal models. Relatively recent studies implicating another protein, TDP-43, in familial and sporadic forms of ALS...

Read the complete abstract on PubMed

Topics

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

Share to Amyotrophic Lateral Sclerosis
Share to Frontotemporal Dementia

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Gene Knock-In Techniques

Share to Intercellular Signaling Peptides and Proteins

Share to Motor Neurons

Share to Peptides

Share to Spinal Cord

Share to Ubiquitin

Source

Open in PubMed Open full text

Identifiers

PMID: 22898872
DOI: 10.1038/cddis.2012.115
PMCID: PMC3434652

Genes

VCP
SOD1

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.PMID 22898872Read ↗

A progressive translational mouse model of human valosin‐containing protein disease: The <i>VCP</i><sup>R155H/+</sup> mouseSemantic proximity Read ↗

The Homozygote VCPR155H/R155H Mouse Model Exhibits Accelerated Human VCP-Associated Disease PathologySemantic proximity Read ↗

Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and boneSemantic proximity Read ↗

Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis.Semantic proximity Read ↗

Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in miceSemantic proximity Read ↗

Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.Semantic proximity Read ↗

Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.Semantic proximity Read ↗

Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1.Semantic proximity Read ↗

Motor neuron involvement in multisystem proteinopathy: implications for ALS.Semantic proximity Read ↗

The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.Semantic proximity Read ↗

Valosin Containing Protein Associated Fronto-Temporal Lobar Degeneration:Clinical Presentation, Pathologic Features and PathogenesisSemantic proximity Read ↗

An Early Onset Progressive Motor Neuron Disorder inScyl1-Deficient Mice Is Associated with Mislocalization of TDP-43Semantic proximity Read ↗

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