Are biological sensors modulated by their structural scaffolds? The role of the structural muscle proteins α-actinin-2 and α-actinin-3 as modulators of biological sensors

Lek, Monkol; North, Kathryn N.

doi:10.1016/j.febslet.2010.05.059

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Volume 584, Issue 14 , Pages 2974-2980, 16 July 2010

Are biological sensors modulated by their structural scaffolds? The role of the structural muscle proteins α-actinin-2 and α-actinin-3 as modulators of biological sensors

Edited by Berend Wieringa

Institute for Neuroscience and Muscle Research, The Children’s Hospital at Westmead, Sydney 2145, NSW, Australia

Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney, Sydney 2006, NSW, Australia

Received 12 March 2010; received in revised form 26 May 2010; accepted 26 May 2010. published online 31 May 2010.

Abstract

Biological sensors and their ability to detect and respond to change in the cellular environment can be modulated by protein scaffolds acting within their interaction network. The skeletal muscle α-actinins have been considered as primarily structural scaffold proteins. However, deficiency of α-actinin-3 due to a common null polymorphism results in predominantly metabolic changes in skeletal muscle function. In this review, we explore the range of phenotypes associated with α-actinin-3 deficiency, and draw supporting evidence from known interaction partners for its role as a scaffold which acts to modulate biological sensors that result in changes in muscle metabolism and structure.

Keywords: Actinin, Skeletal muscle, Structural scaffold, Biological sensor