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脊髓性肌萎缩症致病蛋白 SMN 在巨噬细胞中可能具有什么功能?

What could be the function of the spinal muscular atrophy-causing protein SMN in macrophages?

机构信息

SMATHERIA gGmbH - Non-Profit Biomedical Research Institute, Hannover, Germany.

Center for Systems Neuroscience (ZSN), Hannover, Germany.

出版信息

Front Immunol. 2024 May 28;15:1375428. doi: 10.3389/fimmu.2024.1375428. eCollection 2024.

Abstract

Spinal Muscular Atrophy (SMA), a neurodegenerative disorder, extends its impact beyond the nervous system. The central protein implicated in SMA, Survival Motor Neuron (SMN) protein, is ubiquitously expressed and functions in fundamental processes such as alternative splicing, translation, cytoskeletal dynamics and signaling. These processes are relevant for all cellular systems, including cells of the immune system such as macrophages. Macrophages are capable of modulating their splicing, cytoskeleton and expression profile in order to fulfil their role in tissue homeostasis and defense. However, less is known about impairment or dysfunction of macrophages lacking SMN and the subsequent impact on the immune system of SMA patients. We aimed to review the potential overlaps between SMN functions and macrophage mechanisms highlighting the need for future research, as well as the current state of research addressing the role of macrophages in SMA.

摘要

脊髓性肌萎缩症(SMA)是一种神经退行性疾病,其影响不仅局限于神经系统。在 SMA 中起核心作用的蛋白是运动神经元存活蛋白(SMN),该蛋白广泛表达,并参与多种基本过程,如可变剪接、翻译、细胞骨架动态和信号转导。这些过程与所有细胞系统都相关,包括免疫系统的细胞,如巨噬细胞。巨噬细胞能够调节其可变剪接、细胞骨架和表达谱,以实现其在组织稳态和防御中的作用。然而,对于缺乏 SMN 的巨噬细胞的损伤或功能障碍及其对 SMA 患者免疫系统的后续影响知之甚少。我们旨在综述 SMN 功能与巨噬细胞机制之间的潜在重叠,强调未来研究的必要性,以及当前研究中涉及巨噬细胞在 SMA 中作用的现状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/11165114/345e0f6305ec/fimmu-15-1375428-g001.jpg

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