Intermediate filaments and the function of the dystrophin-protein complex.
Intermediate filament (IF) proteins and the dystrophin-associated protein complex (DPC) play important roles in cardiac and skeletal muscle. Both systems are mutated in several different forms of inherited muscular dystrophy and cardiomyopathy. Recently two articles have been published that propose a physical link between the DPC and the IF network in muscle. Two novel IF proteins, syncoilin and desmuslin, have been identified as binding partners for the dystrophin-associated protein, alpha-dystrobrevin, in muscle. These novel interactions suggest that alpha-dystrobrevin may tether the IF protein network to the DPC. Mice lacking alpha-dystrobrevin develop muscular dystrophy without perturbing the assembly of the DPC at the muscle membrane, suggesting the involvement of other non-DPC proteins in the disease. The interaction between the DPC and the IF network may be disrupted in patients with Duchenne muscular dystrophy and in mice lacking alpha-dystrobrevin.
Blake
Derek J
DJ
Department of Phrmacology, University of Oxford, Oxford, United Kingdom. dblake@enterprise.molbiol.ox.ac.uk
Martin-Rendon
Enca
E
D003598:Cytoskeletal Proteins / Q000502:physiology*; D049030:Dystroglycans; D016189:Dystrophin / Q000502:physiology*; D006801:Humans; D007381:Intermediate Filament Proteins / Q000502:physiology; D008562:Membrane Glycoproteins / Q000502:physiology*; D009124:Muscle Proteins / Q000502:physiology; D009136:Muscular Dystrophies / Q000503:physiopathology*
D003598:Cytoskeletal Proteins; C483081:DAG1 protein, human; D016189:Dystrophin; D007381:Intermediate Filament Proteins; D008562:Membrane Glycoproteins; D009124:Muscle Proteins; C503784:SYNC protein, human; C430456:desmuslin; D049030:Dystroglycans