Although the pathogenesis of ALS is still unknown, extensive studies have revealed important genetic risk factors in the past decade.C9orf72 (#MIM 105550),SOD1 (#MIM105400),TARDBP (#MIM612069, #MIM612069), andFUS (#MIM608030) are the most frequently mutated genes that have been shown in ALS,2,3 with the hexanucleotide repeat expansion inC9orf72 being the most significant and accounting for 30–50% of familial and 7% of sporadic ALS cases in the European population.2,3 However, the genetic basis of ALS has not been investigated in the Transcaucasian region. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.