The Sarcomere and Skeletal Muscle Disease

This book reviews diseases caused by mutated proteins in the different sub-compartments of the sarcomere. It also documents techniques used to investigate the pathobiological bases of the diseases and discusses possible therapeutic options.

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.