Immunodeficiencies Laboratory, National Institute of Pediatrics (INP), Mexico City 04530, Mexico.
Doctorate Program in Biological Sciences, Autonomous National University of Mexico (UNAM), Mexico City 04510, Mexico.
Int J Mol Sci. 2023 Feb 4;24(4):3098. doi: 10.3390/ijms24043098.
Cyclic AMP-dependent protein kinase A (PKA) is a ubiquitous enzymatic complex that is involved in a broad spectrum of intracellular receptor signaling. The activity of PKA depends on A-kinase anchoring proteins (AKAPs) that attach to PKAs close to their substrates to control signaling. Although the relevance of PKA-AKAP signaling in the immune system is evident in T cells, its relevance in B and other immune cells remains relatively unclear. In the last decade, lipopolysaccharide-responsive and beige-like anchor protein (LRBA) has emerged as an AKAP that is ubiquitously expressed in B and T cells, specifically after activation. A deficiency of LRBA leads to immune dysregulation and immunodeficiency. The cellular mechanisms regulated by LRBA have not yet been investigated. Therefore, this review summarizes the functions of PKA in immunity and provides the most recent information regarding LRBA deficiency to deepen our understanding of immune regulation and immunological diseases.
环腺苷酸依赖的蛋白激酶 A(PKA)是一种普遍存在的酶复合物,参与广泛的细胞内受体信号转导。PKA 的活性依赖于 A 激酶锚定蛋白(AKAPs),它们与靠近底物的 PKAs 结合,以控制信号转导。尽管 PKA-AKAP 信号在 T 细胞中的相关性是显而易见的,但它在 B 细胞和其他免疫细胞中的相关性仍然相对不清楚。在过去的十年中,脂多糖反应性和米色样锚蛋白(LRBA)已成为一种 AKAP,它在 B 和 T 细胞中广泛表达,特别是在激活后。LRBA 的缺乏会导致免疫失调和免疫缺陷。LRBA 调节的细胞机制尚未被研究。因此,本综述总结了 PKA 在免疫中的功能,并提供了关于 LRBA 缺乏的最新信息,以加深我们对免疫调节和免疫性疾病的理解。