For discussion of the increased blood concentrations of acylcarnitines and amino acids in homozygous R405W desmin knock‐in mice, which could serve as biomarkers of secondary mitochondrial dysfunction in autosomal‐recessive desminopathies, and the presence of alterations in the UFMylation machinery in hetero‐ and homozygous mice, but the absence of abortive translation during the synthesis of R406W mutant desmin protein, see the ‘Expanded Discussion.’ This evidence concerns the gene DES and Desminopathy.