TDP‐43 liberates mtDNA into the cytoplasm via the mPTP, leading to activation of the cGAS/STING signaling pathway that was found to be pertinent in ALS and liver fibrosis.[26a,e] Mitochondrial dysfunction subsequently activates the mtDNA‐cGAS‐STING pathway in kidney injury.[26c] In our study, mitochondrial content reduction was observed in circTmeff1 overexpressed myotube cells and mice. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.