The Mislocalization of TDP-43 to Mitochondria Impairs Myotube Maturation - PubMed
2 days ago
- #TDP-43
- #ALS
- #mitochondria
- TDP-43 aggregation in neuronal cells is a hallmark of ALS.
- TDP-43 pathology also occurs in skeletal muscle fibers, but its role is not well understood.
- Study used C2C12 myoblast cells to investigate TDP-43 localization during myogenic differentiation.
- TDP-43 translocates to mitochondria as myotubes mature.
- Increased mitochondrial TDP-43 was observed in ALS patient muscle tissues.
- Inhibiting TDP-43 mitochondrial translocation enhances myotube maturation.
- Findings suggest TDP-43 mislocalization affects myogenic differentiation and muscle degeneration in TDP-43 proteinopathies.