Twenty-two individuals across four generations suffer a chromosome 19p13.3-linked autosomal dominant progressive myopathy with distinctive pathology including rimmed ubiquitin-positive autophagic vacuolation [[6]] (Fig. PCR amplification of this exon in patient genomic DNA and muscle RNA revealed the wild-type band, and a second ~ 1000 bp higher band (Fig. Many cases of Inclusion Body Myopathy, the most common of the myopathies, exhibit aggrephagic activation, including NBR1 deposition, not dissimilar to the present patients [[5]]. [Extracted from the article]