Elevated sphingosine-1-phosphate lyase leads to increased metabolism and reduced survival in adrenocortical carcinoma

Adrenocortical carcinomas (ACCs) are invasive tumours arising in the adrenal cortex, and steroidogenic tumours are associated with worse prognostic outcomes. Loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1) cause primary adrenal insufficiency and as a key degradative enzyme in the...

Descripción completa

Detalles Bibliográficos
Autores: Williams, Jack L., Smith, Chris, Hall, Charlotte, Khaled, Zakaa, Maharaj, Avinaash, Kwong, Ruth, Pittaway, James, Casas, Josefina, Parvanta, Laila, Abdel-Aziz, Tarek Ezzat, Palazzo, Fausto, Chung, Teng-Teng, Guasti, Leonardo, Metherell, Lou, Prasad, Rathi
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2023
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/287870
Acceso en línea:http://hdl.handle.net/10261/287870
https://api.elsevier.com/content/abstract/scopus_id/85146412492
Access Level:acceso abierto
Palabra clave:Sphingosine-1-phosphate lyase
SGPL1
Adrenocortical carcinoma
Sphingolipids
Descripción
Sumario:Adrenocortical carcinomas (ACCs) are invasive tumours arising in the adrenal cortex, and steroidogenic tumours are associated with worse prognostic outcomes. Loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1) cause primary adrenal insufficiency and as a key degradative enzyme in the sphingolipid pathway, SGPL1 also influences the balance of pro-proliferative and pro-apoptotic sphingolipids. We, therefore, hypothesized increased SGPL1 may be linked to increased disease severity in ACC.