While the role of amyloid deposits in IBM pathogenesis remains uncertain, the accumulation of TDP-43+ inclusions in the sarcoplasm of IBM patients is accompanied by TDP-43 nuclear depletion, resulting in loss of TDP-43 splicing repression of non-conserved cryptic exons, a feature seen in amyotrophic lateral sclerosis and frontotemporal dementia (77). This evidence concerns the gene TARDBP and frontotemporal dementia.