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

Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis.

PLoS genetics - 1 Sept 2014

Liang Cyong-Jhih, Chang Ya-Chu, Chang Henry C, Wang Chung-Kang, Hung Yu-Chien, Lin Ying-Er, Chan Chia-Ching, Chen Chun-Hong, Chang Hui-Yun, Sang Tzu-Kang

Abstract excerpt

Mutations in VCP (Valosin-containing protein), an AAA ATPase critical for ER-associated degradation, are linked to IBMPFD (Inclusion body myopathy with Paget disease and frontotemporal dementia). Using a Drosophila IBMPFD model, we have identified the ER protein Derlin-1 as a modifier of pathogenic TER94 (the fly VCP homolog) mutants. Derlin-1 binds to TER94 directly, and this interaction is essential for...

Read the complete abstract on PubMed

Topics

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

Share to Adenosine Triphosphatases
Share to Caspases

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Endoplasmic Reticulum-Associated Degradation

Share to Endoplasmic Reticulum Stress

Share to Enzyme Activation

Share to Neurodegenerative Diseases

Share to Protein Interaction Domains and Motifs

Share to Valosin Containing Protein

Source

Open in PubMed Open full text

Identifiers

PMID: 25255315
DOI: 10.1371/journal.pgen.1004675
PMCID: PMC4177747

Genes

VCP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis.PMID 25255315Read ↗

Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.Semantic proximity Read ↗

Mechanisms of Cdc48/VCP-mediated cell death: from yeast apoptosis to human disease.Semantic proximity Read ↗

TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.Semantic proximity Read ↗

Identification of ter94, Drosophila VCP, as a modulator of polyglutamine-induced neurodegeneration.Semantic proximity Read ↗

The VCP/p97 system at a glance: connecting cellular function to disease pathogenesisSemantic proximity Read ↗

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

Valosin-Containing Protein (VCP)/p97 Oligomerization.Semantic proximity Read ↗

Valosin containing protein (VCP): initiator, modifier, and potential drug target for neurodegenerative diseases.Semantic proximity Read ↗

Different dynamic movements of wild‐type and pathogenic <scp>VCP</scp>s and their cofactors to damaged mitochondria in a <scp>P</scp>arkin‐mediated mitochondrial quality control systemSemantic proximity Read ↗

ATPase activity of p97/valosin-containing protein is regulated by oxidative modification of the evolutionally conserved cysteine 522 residue in Walker A motif.Semantic proximity Read ↗

Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration.Semantic proximity Read ↗

Caspar, an adapter for VAPB and TER94, modulates the progression of ALS8 by regulating IMD/NFκB-mediated glial inflammation in a Drosophila model of human disease.Semantic proximity Read ↗

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