The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

• Published in: Human molecular genetics Vol. 22; no. 23; pp. 4706 - 4719
• Main Authors: Wang, Wenzhang, Li, Li, Lin, Wen-Lang, Dickson, Dennis W., Petrucelli, Leonard, Zhang, Teng, Wang, Xinglong
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.12.2013
• Subjects: Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - genetics; Amyotrophic Lateral Sclerosis - pathology; Amyotrophic Lateral Sclerosis - physiopathology; Animals; Cells, Cultured; DNA-Binding Proteins - genetics; DNA-Binding Proteins - physiology; Female; Gene Expression Regulation; Gene Knockdown Techniques; Humans; Male; Membrane Proteins - physiology; Mitochondria - physiology; Mitochondria - ultrastructure; Mitochondrial Dynamics; Mitochondrial Proteins - physiology; Motor Neurons - pathology; Motor Neurons - physiology; Mutation; Neurites - metabolism; Neurites - ultrastructure; Pregnancy; Rats; Rats, Sprague-Dawley; Rats, Transgenic

Mutations in TDP-43 lead to familial ALS. Expanding evidence suggests that impaired mitochondrial dynamics likely contribute to the selective degeneration of motor neurons in SOD1-associated ALS. In this study, we investigated whether and how TDP-43 mutations might impact mitochondrial dynamics and...