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Steroidogenic factor-1 lineage origin of skin lesions in Carney complex syndrome

Archive ouverte | Sahut-Barnola, Isabelle | CCSD

ABSTRACT Carney complex (CNC) is a rare familial multi-neoplastic syndrome predisposing to endocrine and non-endocrine tumors due to inactivating mutations of PRKAR1A leading to perturbations of the cAMP protein kinase A (PKA) sig...

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

Archive ouverte | Djari, Cyril | CCSD

International audience. 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 fo...

Loss of SUMO-specific protease 2 causes isolated glucocorticoid deficiency by blocking adrenal cortex zonal transdifferentiation

Archive ouverte | Dufour, Damien | CCSD

Abstract SUMOylation is a dynamic posttranslational modification, that provides fine-tuning of protein function involved in the cellular response to stress, differentiation, and tissue development. In the adrenal cortex, an emblem...

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