title: Autophagic induction of amyotrophic lateral sclerosis-linked Cu/Zn superoxide dismutase 1 G93A mutant in NSC34 cells.
abstract: Previous studies have confirmed that the beclin 1 complex plays a key role in the initial stage of autophagy and deregulated autophagy might involve in amyotrophic lateral sclerosis. However, the mechanism underlying altered autophagy associated with the beclin 1 complex remains unclear. In this study, we transfected the Cu/Zn superoxide dismutase 1 G93A mutant protein into the motor neuron-like cell line NSC34 cultured in vitro. Western blotting and co-immunoprecipitation showed that the Cu/Zn superoxide dismutase 1 G93A mutant enhanced the turnover of autophagic marker microtubule-associated protein light chain 3II (LC3II) and stimulated the conversion of EGFP-LC3I to EGFP-LC3II, but had little influence on the binding capacity of the autophagy modulators ATG14L, rubicon, UVRAG, and hVps34 to beclin 1 during autophagosome formation. These results suggest that the amyotrophic lateral sclerosis-linked Cu/Zn superoxide dismutase 1 G93A mutant can upregulate autophagic activity in NSC34 cells, but that this does not markedly affect beclin 1 complex components.
authors.affiliation: College of Life Science, Northwest Agriculture & Forestry University, Yangling, Shaanxi Province, China.
authors.forename: Yanming
authors.initials: Y
authors.lastname: Wei
keywords: NSC34 cells; Oversea Study Fellowship from the China Scholarship Council; SOD1 G93A mutant; aggregates; amyotrophic lateral sclerosis; autophagy; beclin 1; beclin 1 interacting proteins; nerve regeneration; neural regeneration; neurodegeneration