Structural and functional aspects of filamins

Filamins are a family of high molecular mass cytoskeletal proteins that org anize filamentous actin in networks and stress fibers. Over the past few ye ars it has become clear that filamins anchor various transmembrane proteins to the actin cytoskeleton and provide a scaffold for a wide range of cytop lasmic signaling proteins. The recent cloning of three human filamins and s tudies on filamin orthologues from chicken and Drosophila revealed unexpect ed complexity of the filamin family, the biological implications of which h ave just started to be addressed. Expression of dysfunctional filamin-A lea ds to the genetic disorder of ventricular heterotopia and gives reason to e xpect that abnormalities in the other isogenes may also be connected with h uman disease. In this review aspects of filamin structure, its splice varia nts, binding partners and biological function will be discussed. (C) 2001 E lsevier Science B.V. All rights reserved.