MYH14 and Skeletal myopathy: In summary, the general model proposed here for proline rod mutations causing skeletal myopathies is based on the following sequence of events: (a) formation of a local kinking of the coiled-coil caused by unique proline structural properties; (b)spreading of the distortion along the sarcomere by myosin staggered assembly; and (c) a shift in the SRX/DRX ratio with a subsequent increase in the muscle metabolic rate as reported for a subset of HCM myosin mutations located in the motor domain (67–70).