Inflammation, fibrosis and skeletal muscle regeneration in LGMDR9 are orchestrated by macrophages,Neuropathology and Applied Neurobiology

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Inflammation, fibrosis and skeletal muscle regeneration in LGMDR9 are orchestrated by macrophages
Neuropathology and Applied Neurobiology ( IF 4.0 ) Pub Date : 2021-05-10 , DOI: 10.1111/nan.12730
Heike Kölbel ₁ , Corinna Preuße _{2,

3} , Lukas Brand ₁ , Arpad von Moers ₄ , Adela Della Marina ₁ , Markus Schuelke ₅ , Andreas Roos ₁ , Hans-Hilmar Goebel _{2,

6} , Ulrike Schara-Schmidt ₁ , Werner Stenzel ₂

Affiliation

Variable degrees of inflammation, necrosis, regeneration and fibrofatty replacement are part of the pathological spectrum of the dystrophic process in alpha dystroglycanopathy LGMDR9 (FKRP-related, OMIM #607155), one of the most prevailing types of LGMDs worldwide. Inflammatory processes and their complex interplay with vascular, myogenic and mesenchymal cells may have a major impact on disease development. The purpose of our study is to describe the specific immune morphological features in muscle tissue of patients with LGMDR9 to enable a better understanding of the phenotype of muscle damage leading to disease progression.

中文翻译：

LGMDR9 中的炎症、纤维化和骨骼肌再生由巨噬细胞协调

不同程度的炎症、坏死、再生和纤维脂肪替代是 α 肌营养不良症 LGMDR9（FKRP 相关，OMIM #607155）中营养不良过程的病理谱的一部分，这是全球最流行的 LGMD 类型之一。炎症过程及其与血管、肌源性和间充质细胞的复杂相互作用可能对疾病发展产生重大影响。我们研究的目的是描述 LGMDR9 患者肌肉组织中特定的免疫形态学特征，以便更好地了解导致疾病进展的肌肉损伤表型。

更新日期：2021-05-10

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