DES and Desminopathy: When reflecting the overall clinical and myopathological similarities as well as discrepancies between our het and hom R349P desmin knock-in models and human desminopathy patients, homozygous R349P desmin knock-in mice, which serve as a model for autosomal-recessive desminopathies, share more commonalities with the human pathology than their heterozygous littermates.