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The effects of SMA are not limited to the nervous system but also encompass multiple cell types. Fibroblasts have been extensively employed as primary disease model cells in SMA pathophysiological studies. Here, we present a comprehensive summary of the pivotal roles fibroblasts play in SMA research, focusing on how SMN deficiency modulates the response characteristics of fibroblasts. Our findings reveal distinct reactivity patterns in fibroblasts, which serve as representative Non\u2010neuronal cells, compared to motor neurons in SMA. This review underscores the crucial roles of fibroblasts in elucidating mechanistic changes, advancing drug discovery, and identifying reliable biomarkers for SMA. 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