Year: 2012
Type: book-chapter
Abstract: Disorders 2proteins that bind to the ECM, and of cytoplasmic proteins that connect to the cytoskeleton and transmit biochemical signals.The DPC is composed of several proteins, which include and -dystroglycan that bind to laminin, dystrophin that connects to the cytoskeleton, and associated proteins such as sarcoglycans and neuronal nitric oxide synthase (nNOS).These proteins have the important function to confer mechanical integrity to the plasma membrane, which otherwise would break following muscle contraction.Indeed, this occurs in patients with mutations in DPC components, and present with several types of severe muscle disease, including Duchenne Muscular Dystrophy (DMD) and various forms of Limb Girdle Muscular Dystrophy (Bushby, 1999;Barresi and Campbell, 2006).While integrins also establish a connection between the ECM and cytoskeletal and signalling proteins, the two complexes are biochemically distinct.As we will see below, integrins appear dispensable for the mechanical integrity of the sarcolemma, but have important functions during all stages of muscle development.
Source: InTech eBooks
DOI: 10.5772/47905
Authors: Susan C., Ulrich Mueller, Francesco J.
Institutions: Scripps Research Institute, University College London
Language: English
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: hybrid
Funder: Muscular Dystrophy Association
OpenAlex ID: W1508701953