Department of Dermatology and Venereology, Innsbruck Medical University, Innsbruck, Austria;
Blood. 2014 Jan 9;123(2):217-27. doi: 10.1182/blood-2013-08-518555. Epub 2013 Oct 3.
Langerhans cells (LCs) are dendritic cells (DCs) residing in epithelia, where they critically regulate immunity and tolerance. The p14 adaptor molecule is part of the late endosomal/LAMTOR (lysosomal adaptor and mitogen-activated protein kinase and mammalian target of rapamycin [mTOR] activator/regulator) complex, thereby contributing to the signal transduction of the extracellular signaling-regulated kinase (ERK) and the mTOR cascade. Furthermore, p14 represents an important regulator for endosomal sorting processes within the cell. Mutated, dysfunctional p14 leads to a human immunodeficiency disorder with endosomal/lysosomal defects in immune cells. Because p14 participates in the regulation of endosomal trafficking, growth factor signaling, and cell proliferation, we investigated the role of p14 in mouse DCs/LCs using a conditional knockout mouse model. p14-deficient animals displayed a virtually complete loss of LCs in the epidermis early after birth due to impaired proliferation and increased apoptosis of LCs. Repopulation analysis after application of contact sensitizer leads to the recruitment of a transient LC population, predominantly consisting of short-term LCs. The underlying molecular mechanism involves the p14-mediated disruption of the LAMTOR complex which results in the malfunction of both ERK and mTOR signal pathways. Hence, we conclude that p14 acts as a novel and essential regulator of LC homeostasis in vivo.
郎格汉斯细胞 (LCs) 是位于上皮组织中的树突状细胞 (DCs),它们在上皮组织中对免疫和耐受起着关键的调节作用。p14 衔接分子是晚期内体/LAMTOR(溶酶体衔接子和丝裂原活化蛋白激酶以及哺乳动物雷帕霉素靶蛋白 [mTOR] 激活剂/调节剂)复合物的一部分,从而有助于细胞外信号调节激酶 (ERK) 和 mTOR 级联反应的信号转导。此外,p14 代表细胞内内体分选过程的重要调节剂。突变的、功能失调的 p14 会导致免疫细胞中内体/溶酶体缺陷的人类免疫缺陷疾病。由于 p14 参与内体运输、生长因子信号和细胞增殖的调节,我们使用条件性敲除小鼠模型研究了 p14 在小鼠 DCs/LCs 中的作用。p14 缺陷动物在出生后早期表皮中的 LCs 几乎完全缺失,这是由于 LCs 增殖受损和凋亡增加所致。接触致敏剂应用后的再群体分析导致短暂的 LCs 群体的募集,主要由短期 LCs 组成。潜在的分子机制涉及 p14 介导的 LAMTOR 复合物的破坏,导致 ERK 和 mTOR 信号通路的功能障碍。因此,我们得出结论,p14 作为体内 LC 动态平衡的新型和必需调节剂。