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.
Standards are all around us, even if we are not always aware of them. One example of a widely-used standard is the A4 size for sheets of paper. A standard is a document that sets out requirements for a specific item, material, component, system or service, or describes in detail a particular method or procedure.Standards facilitate international trade by ensuring compatibility and interoperability of components, products and services.herefore, a European Standard (EN) automatically becomes a national standard in each of the 33 CEN-CENELEC member countries. Standards are voluntary which means that there is no automatic legal obligation to apply them. However, laws and regulations may refer to standards and even make compliance with them compulsory.