Transcriptomic profiling of symptomatic and end-stage SOD1-G93A transgenic mice reveals extracellular matrix components as key players in ALS pathogenesis

IF 4.2 2区生物学 Q2 BIOCHEMISTRY & MOLECULAR BIOLOGY Biochimica et biophysica acta. Molecular basis of disease Pub Date : 2025-02-06 DOI:10.1016/j.bbadis.2025.167707

Simona Rossi , Martina Milani , Ilaria Della Valle , Savina Apolloni

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引用次数: 0

Abstract

•
ALS is a neurodegenerative disease marked by the loss of motor neurons.
•
Damaged neurons are not regenerated but attract immune cells, fibroblasts, and astrocytes.
•
Research suggests fibrosis and inflammation contribute to ALS progression.
•
This study profiled spinal cord tissues from SOD1-G93A male mice at different disease stages using RNA sequencing.
•
We identified differentially expressed genes and pathways linked to disease progression.
•
We highlighted notable changes in ECM-related genes: Fmod, S100a4, S100a6 and Col1a1.
•
These findings provide insights into ALS pathology and potential therapeutic targets.

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有症状和终末期SOD1-G93A转基因小鼠的转录组学分析揭示了细胞外基质成分在ALS发病机制中的关键作用。

肌萎缩侧索硬化症是一种以运动神经元丧失为特征的神经退行性疾病。•受损的神经元不能再生，但会吸引免疫细胞、成纤维细胞和星形胶质细胞。•研究表明，纤维化和炎症有助于ALS的进展。•本研究利用RNA测序分析了SOD1-G93A雄性小鼠在不同疾病阶段的脊髓组织。•我们确定了与疾病进展相关的差异表达基因和途径。•我们强调了ecm相关基因的显著变化：Fmod， S100a4， S100a6和Col1a1。•这些发现为ALS的病理和潜在的治疗靶点提供了见解。

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

Biochimica et biophysica acta. Molecular basis of disease 生物-生化与分子生物学

CiteScore

12.30

自引率

0.00%

发文量

218

审稿时长

32 days

期刊介绍： BBA Molecular Basis of Disease addresses the biochemistry and molecular genetics of disease processes and models of human disease. This journal covers aspects of aging, cancer, metabolic-, neurological-, and immunological-based disease. Manuscripts focused on using animal models to elucidate biochemical and mechanistic insight in each of these conditions, are particularly encouraged. Manuscripts should emphasize the underlying mechanisms of disease pathways and provide novel contributions to the understanding and/or treatment of these disorders. Highly descriptive and method development submissions may be declined without full review. The submission of uninvited reviews to BBA - Molecular Basis of Disease is strongly discouraged, and any such uninvited review should be accompanied by a coverletter outlining the compelling reasons why the review should be considered.