The Sarcomere and Sarcomerogenesis -- Skeletal Muscle Alpha-Actin Diseases -- Nebulin-A Giant Chameleon -- Skeletal Muscle Disease Due to Mutations in Tropomyosin, Troponin and Cofilin -- Investigations into the Pathobiology of Thin-Filament Myopathies -- Mouse Models for Thin Filament Disease -- Thick Filament Diseases -- Acute Quadriplegic Myopathy: An Acquired "Myosinopathy" -- Third Filament Diseases -- The Z-Disk Diseases -- Intermediate Filament Diseases: Desminopathy -- Muscular Integrity-A Matter of Interlinking Distinct Structures via Plectin -- The Sarcomere and the Nucleus: Functional Links to Hypertrophy, Atrophy and Sarcopenia -- Other Model Organisms for Sarcomeric Muscle Diseases -- Therapeutic Approaches for the Sarcomeric Protein Diseases. Although best known for its role in heart disease, the sarcomere--the fundamental unit of muscle contraction--is also involved in skeletal muscle diseases. Chapters in The Sarcomere and Skeletal Muscle Disease provide an up-to-date review of diseases caused by mutated proteins in the different sub-compartments of the sarcomere, document the techniques currently being used to investigate the pathobiological bases of the diseases, which remain largely unknown, and discuss possible therapeutic options.