Article
Pseudokinase-converting mutation in protein kinase C alpha drives chordoid glioma by pathway rewiring.
Proceedings of the National Academy of Sciences of the United States of America - 21 Jul 2026
Bellamy Charlotte, Tovell Hannah, Kao Tiffany H, Kornev Alexandr, Letourneur Quentin, Arslan Janan, Schwaighofer Selina, Baffi Timothy R, Dingli Florent, Loew Damarys, Lerond Julie, Liva Stephane, Izac Brigitte, Andrieu Muriel, Adle-Biassette Homa, Barnier Jean-Vianney, Stefan Eduard, Taylor Susan S, Sanson Marc, Newton Alexandra C, Bielle Franck
Abstract excerpt
Chordoid glioma (ChG) is a rare, low-grade brain tumor characterized by a novel recurrent point mutation, D463H, in the kinase domain of protein kinase C alpha (PKCα). The mutation is invariably an Asp to His substitution, suggesting a unique function beyond catalytic inactivation associated with other cancer-associated PKCα mutations. Here, we show that this mutation converts PKCα into a pseudokinase, abolishing...
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