Walton and Nattrass first proposed limb-girdle muscular dystrophy (LGMD) as a nosological entity in 1954.Their definition included the following characteristics:, 20. Schematic of the sarcomere with labeled molecular components that are known to cause limb-girdle muscular dystrophy or myofibrillar myopathy. Mutations in actin and nebulin cause the congenital myopathy nemaline rod myopathy, and the mutations in myosin cause familial hypertrophic cardiomyopathy.
In Lean, Standard Work (often called standardized work) is the cornerstone of any continuous improvement effort. It locks in gains and provides a foundation for future advances. Standard Work helps companies reach their improvement targets, but also provides a stable, reasonable working environment for frontline employees.
For direct labor, the standard cost is the amount per hour the employee earns plus the cost to the employer for employment taxes and benefits. The standard labor cost per part is calculated by taking the labor cost per hour divided by the parts per hour the employee is able to produce.