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免疫遗传缺陷中的体细胞嵌合现象。

Somatic mosaicism in genetic errors of immunity.

作者信息

Cooper Megan A

机构信息

Department of Pediatrics, Division of Rheumatology/Immunology, Washington University in St Louis, St Louis, Mo.

出版信息

J Allergy Clin Immunol. 2025 Mar;155(3):759-767. doi: 10.1016/j.jaci.2024.11.038. Epub 2024 Dec 24.

Abstract

Genetic mosaicism in somatic cells can lead to the presence of pathogenic variants in a subset of immune cells causing genetic errors of immunity, often phenocopying germline inborn errors of immunity. Over the last 2 decades, significant progress has been made in the identification of these disorders in patients, including discovery of new diseases. Diagnosis of disease-causing somatic mosaicism provides a target for treatment and monitoring of patients and has implications for genetic counseling. However, there continue to be barriers in the identification of somatic mosaicism, particularly for the clinical diagnosis of patients, based on the limitations of current diagnostic sequencing and analysis approaches. This review focuses on how somatic mosaicism can lead to genetic errors of immunity, the genes known to be associated with somatic genetic errors of immunity, and challenges in the field for accurate diagnosis of patients.

摘要

体细胞中的基因嵌合现象可导致一部分免疫细胞中出现致病变异,从而引发免疫方面的基因错误,这常常表现为与种系遗传性免疫缺陷相似的表型。在过去20年里,在识别这些患者的疾病方面取得了重大进展,包括发现了一些新疾病。致病体细胞嵌合现象的诊断为患者的治疗和监测提供了靶点,并且对遗传咨询具有重要意义。然而,基于当前诊断测序和分析方法的局限性,在识别体细胞嵌合现象方面仍然存在障碍,尤其是在患者的临床诊断方面。本综述重点关注体细胞嵌合现象如何导致免疫方面的基因错误、已知与体细胞免疫基因错误相关的基因,以及该领域在准确诊断患者方面所面临的挑战。

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本文引用的文献

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Somatic mutations in autoinflammatory and autoimmune disease.自身炎症性和自身免疫性疾病中的体细胞突变。
Nat Rev Rheumatol. 2024 Nov;20(11):683-698. doi: 10.1038/s41584-024-01168-8. Epub 2024 Oct 11.

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