Low autophagy capacity implicated in motor system vulnerability to mutant superoxide dismutase

• Published in: Acta neuropathologica communications Vol. 4; no. 1; p. 6
• Main Authors: Tokuda, Eiichi, Brännström, Thomas, Andersen, Peter M., Marklund, Stefan L.
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 25.01.2016; BioMed Central
• Subjects: Adult; Aged; Aged, 80 and over; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - genetics; Amyotrophic Lateral Sclerosis - pathology; Animals; Apoptosis Regulatory Proteins - deficiency; Apoptosis Regulatory Proteins - genetics; Autophagy; Autophagy (Cytology); Autophagy - genetics; Beclin-1; C9orf72 Protein; Disease Models, Animal; Female; Gene expression; Gene Expression Regulation - genetics; Genetic aspects; Humans; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Middle Aged; Motor ability; Motor system vulnerability; Mutation - genetics; Parkinson Disease - genetics; Parkinson Disease - pathology; Phenotype; Physiological aspects; Properties; Proteasome Endopeptidase Complex - metabolism; Protein aggregates; Protein Aggregation, Pathological - genetics; Proteins - genetics; Spinal Cord - metabolism; Spinal Cord - pathology; Superoxide dismutase; Superoxide Dismutase - genetics; Superoxide sumutase-1; Ubiquitinated Proteins - genetics; Ubiquitinated Proteins - metabolism

The motor system is selectively vulnerable to mutations in the ubiquitously expressed aggregation-prone enzyme superoxide dismutase-1 (SOD1). Autophagy clears aggregates, and factors involved in the process were analyzed in multiple areas of the CNS from human control subjects (n = 10) and amyotroph...