FUS and amyotrophic lateral sclerosis: Subsequently, we investigated various disease-relevant models with FUS mutations/proteinopathy, such as ALS patient-derived cell lines with FUS mutations, their mutation-corrected isogenic control lines, autopsied ALS patient spinal cord tissues, as well as a human FUS R495X (hFUS*R495X) transgenic mouse model to uncover the implications of compromised FUS functions in causing mtDNA damage and overall mitochondrial dysfunction in FUS-associated ALS.