This co-expression suggests a known regulatory triad in which USP30-mediated deubiquitination stabilizes Mfn2 to suppress mitophagy, while AMBRA1 provides a compensatory mechanism to restore mitochondrial clearance [41], indicating a dynamic balance between fusion, degradation, and autophagy which could be relevant for mitochondrial integrity in the early DM1 pathology. This evidence concerns the gene USP30 and myotonic dystrophy type 1.