MRAP deficiency impairs adrenal progenitor cell differentiation and gland zonation.

ABSTRACT: Melanocortin 2 receptor accessory protein (MRAP) is a single transmembrane domain accessory protein and a critical component of the hypothamo-pituitary-adrenal axis. MRAP is highly expressed in the adrenal gland and is essential for adrenocorticotropin hormone (ACTH) receptor expression and function. Human loss-of-function mutations in MRAP cause familial glucocorticoid (GC) deficiency (FGD) type 2 (FGD2), whereby the adrenal gland fails to respond to ACTH and to produce cortisol. In this study, we generated Mrap-null mice to study the function of MRAP in vivo. We found that the vast majority of Mrap^-/- mice died at birth but could be rescued by administration of corticosterone to pregnant dams. Surviving Mrap^-/- mice developed isolated GC deficiency with normal miner

SUBMITTER: Novoselova TV 

PROVIDER: S-EPMC6181639 | biostudies-literature | 2018 Jun

REPOSITORIES: biostudies-literature