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Article

Loss of function of VCP/TER94 causes neurodegeneration.

Disease models & mechanisms - 1 Dec 2024

Tsumaki Kohei, Bertens Christian J F, Nakayama Minoru, Kato Saya, Jonao Yuki, Kuribayashi Ayu, Sato Konosuke, Ishiyama Shota, Asakawa Momoko, Aihara Riko, Yoshioka Yuki, Homma Hidenori, Tanaka Hikari, Fujita Kyota, Okazawa Hitoshi, Sone Masaki

Abstract excerpt

Variants in several genes are linked to human frontotemporal lobar degeneration (FTLD) associated with TDP43- and/or ubiquitin-positive inclusions. However, it is not yet clear whether the underlying mechanism is a gain-of-function or a loss-of-function one. To answer this question, we used Drosophila expressing double-stranded RNA against the FTLD-associated gene TER94 (an ortholog of VCP/p97) and found that the...

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Source

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Identifiers

PMID: 39629589
DOI: 10.1242/dmm.050359
PMCID: PMC11698056

Genes

VCP
TARDBP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 23 Oct 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Loss of function of VCP/TER94 causes neurodegeneration.PMID 39629589Read ↗

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

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

TER94 , the Drosophila Homologue of the ALS-related VCP gene, Influences Lifespan and Leads to a Decline in Motor FunctionSemantic proximity Read ↗

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

Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS.Semantic proximity Read ↗

Neuronal function and dysfunction of Drosophila dTDP.Semantic proximity Read ↗

TDP-43 loss-of-function causes neuronal loss due to defective steroid receptor-mediated gene program switching in Drosophila.Semantic proximity Read ↗

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

VCP/p97 inhibitor CB-5083 modulates muscle pathology in a mouse model of VCP inclusion body myopathy.Semantic proximity Read ↗

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

VCP functions to maintain nuclear size by managing DNA damage-induced MDC1–p53–autophagy axisSemantic proximity Read ↗

Neuronal VCP loss of function recapitulates FTLD-TDP pathology.Semantic proximity Read ↗

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