The pathogenic mechanism of this gene mutation may be inhibition of proper HSP90AB1 function resulting in the accumulation of TDP-43, destabilization of interactions with other J-proteins involved in neuroprotection, or the inability to accurately chaperone the variety of Hsp70s or Hsp90s involved in ALS-associated protein aggregate (Dilliott et al., 2022). This evidence concerns the gene HSP90AB1 and amyotrophic lateral sclerosis.