Chapter

The Sarcomere and Skeletal Muscle Disease

Volume 642 of the series Advances in Experimental Medicine and Biology pp 1-14

The Sarcomere and Sarcomerogenesis

  • Elisabeth EhlerAffiliated withCardiovascular Division and Randall Division for Cell and Molecular Biophysics, King’s College London Email author 
  • , Mathias GautelAffiliated withCardiovascular Division and Randall Division for Cell and Molecular Biophysics, King’s College London

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Abstract

Striated muscle owes its name to the microscopic appearance, caused by the longitudinal alignment of thousands of highly ordered contractile units, the sarcomeres. The assembly (and disassembly) of these multiprotein complexes (sarcomere assembly or sarcomerogenesis) follows ordered pathways, which are regulated on the transcriptional, translational and posttranslational level. Furthermore, myofibril assembly involves the participation of transient scaffolds and adaptors, notably the microtubule network. Studies in cell culture and developing embryos have revealed common pathways of sarcomere assembly in heart and skeletal muscle. Disruptions in these pathways are implicated in muscle diseases.