Mutations in m>UBQLN2m> have been shown to be a cause of dominant X-linked amyotrophic lateral sclerosis (ALS). Occurrences of mutations in this gene vary across ALS populations. We screened m>UBQLN2m> for mutations in a final cohort of 150 Irish ALS patients. Individuals who were from families with male-to-male transmission or who carried pathogenic hexanucleotide repeat expansions in m>C9orf72m> were excluded. Apart from common synonymous variation, no sequence variants in m>UBQLN2m> were observed. Mutations in m>UBQLN2m> are therefore not a frequent cause of ALS in the Irish population.