Muscle inflammatory pattern in alpha- and gamma-sarcoglycanopathies.

IF 0.8 4区医学 Q4 CLINICAL NEUROLOGY

Clinical Neuropathology Pub Date : 2021-11-01 DOI:10.5414/NP301393

Chiara Panicucci, Serena Baratto, Lizzia Raffaghello, Paola Tonin, Adele D'Amico, Giorgio Tasca, Monica Traverso, Chiara Fiorillo, Carlo Minetti, Stefano Carlo Previtali, Elena Pegoraro, Claudio Bruno

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引用次数: 3

Abstract

Aim: Since the immune system plays a role in the pathogenesis of several muscular dystrophies, we aim to characterize several muscular inflammatory features in α- (LGMD R3) and γ-sarcoglycanopathies (LGMD R5).

Materials and methods: We explored the expression of major histocompatibility complex class I molecules (MHCI), and we analyzed the composition of the immune infiltrates in muscle biopsies from 10 patients with LGMD R3 and 8 patients with LGMD R5, comparing the results to Duchenne muscular dystrophy patients (DMD).

Results: A consistent involvement of the immune response was observed in sarcoglycanopathies, although it was less evident than in DMD. LGMD R3-R5 and DMD shared an abnormal expression of MHCI, and the composition of the muscular immune cell infiltrate was comparable.

Conclusion: These findings might serve as a rationale to fine-tune a disease-specific immunomodulatory regimen, particularly relevant in view of the rapid development of gene therapy for sarcoglycanopathies.

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-和-肌糖病变的肌肉炎症模式。

目的:由于免疫系统在几种肌肉营养不良的发病机制中起作用，我们的目的是表征α- (LGMD R3)和γ-肌糖病(LGMD R5)的几种肌肉炎症特征。材料与方法:我们检测了主要组织相容性复合体I类分子(MHCI)的表达，分析了10例LGMD R3患者和8例LGMD R5患者肌肉活检组织中免疫浸润的组成，并与杜氏肌营养不良患者(DMD)进行了比较。结果:在肌糖病变中观察到一致的免疫反应，尽管不像在DMD中那么明显。LGMD R3-R5与DMD均存在MHCI异常表达，且肌免疫细胞浸润成分具有可比性。结论:这些发现可以作为微调疾病特异性免疫调节方案的基本原理，特别是考虑到肌糖病基因治疗的快速发展。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Neuropathology 医学-病理学

CiteScore

1.60

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： Clinical Neuropathology appears bi-monthly and publishes reviews and editorials, original papers, short communications and reports on recent advances in the entire field of clinical neuropathology. Papers on experimental neuropathologic subjects are accepted if they bear a close relationship to human diseases. Correspondence (letters to the editors) and current information including book announcements will also be published.