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.
Leader Standard Work Definition, Example, Template, and Advice. Leader Standard Work is a critical step in ensuring that the Lean Culture in your organization prospers. But, Leader Standard Work is dependent on a few things. In what follows, Iâll cover a few of those items and provide an example of Leader Standard Work. For a primer on the importance of Standard Work, check out the Standard Pig Game, an interactive game that teaches the importance of Standardized Work. >. Leader Standard Work is meant to accomplish the following: