Institut de Pharmacologie Moléculaire et Cellulaire, CNRS UMR6097, 660 route des Lucioles, Sophia Antipolis, 06560 Valbonne, France.
Best Pract Res Clin Endocrinol Metab. 2010 Dec;24(6):853-64. doi: 10.1016/j.beem.2010.10.009.
During the early phases of development, adrenal glands share a common origin with kidneys and gonads. The action of diverse transcription factors, signalling pathways and endocrine signals is required for the individualization of the adrenal primordium and its subsequent differentiation into an adult adrenal gland, with massive remodelling taking place around the time of birth in humans. Here I summarize the most important steps by which the adrenal cortex is shaped and present an overview of the current understanding of the genes and molecular pathways implicated in adrenal development and involved in the pathogenesis of its congenital diseases. Evidence is accumulating that some pivotal factors acting during adrenocortical development also play an important role to regulate the growth of adrenocortical tumors, representing promising therapeutical targets for a biology-oriented therapy.
在发育的早期阶段，肾上腺与肾脏和性腺具有共同的起源。各种转录因子、信号通路和内分泌信号的作用对于肾上腺原基的个体化及其随后分化为成人肾上腺是必需的，在人类中，大约在出生时发生大规模重塑。在这里，我总结了塑造肾上腺皮质的最重要步骤，并概述了目前对涉及肾上腺发育和先天性疾病发病机制的基因和分子途径的理解。越来越多的证据表明，在肾上腺皮质发育过程中起关键作用的一些因素也在调节肾上腺皮质肿瘤的生长中起着重要作用，这代表了一种基于生物学的治疗方法的有前途的治疗靶点。
