The chordoid glioma PRKCA D463H mutation is a kinase inactive, gain-of-function allele that induces early-onset chondrosarcoma in mice.

doi:10.1126/scisignal.adr0235

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Article

The chordoid glioma PRKCA D463H mutation is a kinase inactive, gain-of-function allele that induces early-onset chondrosarcoma in mice.

Science signaling - 4 Nov 2025

Calleja Véronique, Henry Jack C, Cobbaut Mathias, Sewell Joanne, Rizzoti Karine, Houghton Francesca, Boeing Stefan, Anyanwu Nneka, Varsani-Brown Sunita, Snoeks Thomas, Suárez-Bonnet Alejandro, Priestnall Simon L, McDonald Neil Q, Cameron Angus J M, Parker Peter J

Abstract excerpt

The penetrant D463H mutation in PRKCA, which encodes the kinase PKCα, is a biomarker and driver of chordoid glioma, a type of brain cancer. Here, we found that heterozygous knock-in expression of the D463H mutant in mice elicited the development of chondrosarcomas. The mutant protein kinase was catalytically inactive, but no such oncogenic phenotype was observed for the related inactivating mutation D463N,...

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Topics

Animals
Protein Kinase C-alpha
Mice
Chondrosarcoma
Gain of Function Mutation

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

Humans

Alleles

Glioma

Brain Neoplasms

Cell Line, Tumor

Gene Expression Regulation, Neoplastic

Mutation

Gene Knock-In Techniques

Source

Open in PubMed Open full text

Identifiers

PMID: 41187221
DOI: 10.1126/scisignal.adr0235
PMCID: PMC7618404

Genes

PRKCA
BRD4
MYC

Variants

Provenance

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

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