Protein kinase A drives paracrine crisis and WNT4-dependent testis tumor in Carney complex.

doi:10.1172/JCI146910

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Article

Protein kinase A drives paracrine crisis and WNT4-dependent testis tumor in Carney complex.

The Journal of clinical investigation - 1 Dec 2021

Djari Cyril, Sahut-Barnola Isabelle, Septier Amandine, Plotton Ingrid, Montanier Nathanaëlle, Dufour Damien, Levasseur Adrien, Wilmouth James, Pointud Jean-Christophe, Faucz Fabio R, Kamilaris Crystal, Lopez Antoine-Guy, Guillou Florian, Swain Amanda, Vainio Seppo J, Tauveron Igor, Val Pierre, Lefebvre Hervé, Stratakis Constantine A, Martinez Antoine, Lefrançois-Martinez Anne-Marie

Abstract excerpt

Large-cell calcifying Sertoli cell tumors (LCCSCTs) are among the most frequent lesions occurring in male Carney complex (CNC) patients. Although they constitute a key diagnostic criterion for this rare multiple neoplasia syndrome resulting from inactivating mutations of the tumor suppressor PRKAR1A, leading to unrepressed PKA activity, LCCSCT pathogenesis and origin remain elusive. Mouse models targeting Prkar1a...

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Identifiers

PMID: 34850745
DOI: 10.1172/JCI146910
PMCID: PMC8631603

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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