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.
Technical standard. A technical standard is an established norm or requirement in regard to technical systems. It is usually a formal document that establishes uniform engineering or technical criteria, methods, processes and practices. In contrast, a custom, convention, company product, corporate standard, and so forth that becomes generally accepted and dominant is often called a de facto standard. A technical standard may be developed privately or unilaterally, for example by a corporation, regulatory body, mili