ALS-linked mutant TDP-43 in oligodendrocytes induces oligodendrocyte damage and exacerbates motor dysfunction in mice.

Abstract excerpt

Nuclear clearance and cytoplasmic aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) are pathological hallmarks of amyotrophic lateral sclerosis (ALS) and its pathogenic mechanism is mediated by both loss-of-function and gain-of-toxicity of TDP-43. However, the role of TDP-43 gain-of-toxicity in oligodendrocytes remains unclear. To investigate the impact of excess TDP-43 on oligodendrocytes, we established...

Topics

• Animals
• Oligodendroglia
• DNA-Binding Proteins
• Mice, Transgenic
• Mice
• Amyotrophic Lateral Sclerosis
• Mutation
• Spinal Cord
• Mice, Inbred C57BL