Revival of autochthonous adrenocortical stem cells in autoimmune Addison's disease

肾上腺皮质产生两种类固醇激素，皮质醇和醛固酮，这是生命所必需的。肾上腺皮质的活动，包括皮质醇和醛固酮的产生，是由一种被称为促肾上腺皮质激素（ACTH）的主要调节激素控制的。过多的促肾上腺皮质激素会导致肾上腺扩张并产生过多的皮质醇。相反，过少的ACTH会导致肾上腺萎缩，并由于类固醇缺乏而导致严重的疾病。肾上腺皮质的所有细胞都是从位于肾上腺外缘周围的亲本干细胞中分裂出来的。这些亲本干细胞的增殖和生长也受到同一种激素ACTH的强烈调节。未经治疗的自身免疫性艾迪生？S是一种致命的疾病。肾上腺皮质被免疫系统攻击破坏，导致重要的类固醇激素皮质醇和醛固酮缺乏。在Addison？在这种疾病中，身体会感觉到类固醇激素的缺乏，因此血液中ACTH的水平会变得非常高（通常是正常水平的20倍）。这是身体刺激肾上腺细胞分裂并产生更多必需的类固醇激素的一种尝试。然而，一旦确诊并使用类固醇片替代治疗，ACTH水平就会下降。至关重要的是，这会导致任何剩余的肾上腺干细胞功能的恶化和功能肾上腺组织的进一步减少。目前项目的想法是将ACTH用于治疗自身免疫性Addison的患者。以刺激他们肾上腺中的干细胞恢复活动。ACTH的治疗最初是每天注射，持续10周。这可能最终导致肾上腺功能的恢复和Addison？然后就可以停止服用类固醇药物。他们的病情是可以治愈的。

The cortex of the adrenal gland manufactures two steroid hormones, cortisol and aldosterone, that are essential for life. The activity of the adrenal cortex, including cortisol and aldosterone production, is controlled by a master regulatory hormone known as ACTH (adrenocorticotrophic hormone). Too much ACTH causes the adrenal glands to expand and to make too much cortisol. In contrast, too little ACTH causes the adrenals to shrink, and results in serious illness owing to steroid deficiency. All the cells of the adrenal cortex start life by dividing from their parent stem cells which are located around the outer edge of the adrenal gland. The proliferation and growth of these parent stem cells is also strongly regulated by the same hormone, ACTH. Untreated, autoimmune Addison?s disease is an invariably fatal condition. The adrenal cortex becomes destroyed by an immune system attack, leading to deficiency of the vital steroid hormones, cortisol and aldosterone. During the development of Addison?s disease, the body senses the steroid hormone deficiency, so blood ACTH levels become very high (typically 20-times higher than normal). This is an attempt by the body to stimulate the adrenal cells to divide and make more of the essential steroid hormones. However, once diagnosed and treated with replacement steroid tablets, the ACTH levels fall. Critically, this leads to a worsening of the function of any remaining adrenal stem cells and further reduction in functioning adrenal tissue. The idea of the current project is to give back ACTH to people with treated autoimmune Addison?s disease in order to stimulate the stem cells in their adrenal glands back into activity. The treatment with ACTH would initially be by daily injection for 10 weeks. This could ultimately lead to recovery of adrenal gland function and people with Addison?s disease may then be able to stop their steroid medications. Their condition could be cured.