MRAP deficiency impairs adrenal progenitor cell differentiation and gland zonation

What is it about?

Adrenal gland hormones control a wide range of physiological processes and are vital for life and well-being. The organization of the adrenal gland into concentric zones is essential for correct gland function. How stem or progenitor cells create and maintain these zones is unclear. We previously showed that mutations in MRAP cause familial glucocorticoid deficiency (FGD), a life-threatening condition characterized by isolated glucocorticoid deficiency. Here we have generated Mrap deficient mouse, the first model of isolated glucocorticoid deficiency. These mice have impaired progenitor cell differentiation and adrenal zonation, partly rescuable with glucocorticoids. The study therefore demonstrates a critical role of MRAP in adrenal gland zonation and the potential for regulating the progenitor cell niche in FGD and other conditions requiring glucocorticoid treatment.

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