Structural and functional aspects of filamins

Biochim Biophys Acta. 2001 Apr 23;1538(2-3):99-117. doi: 10.1016/s0167-4889(01)00072-6.

Abstract

Filamins are a family of high molecular mass cytoskeletal proteins that organize filamentous actin in networks and stress fibers. Over the past few years it has become clear that filamins anchor various transmembrane proteins to the actin cytoskeleton and provide a scaffold for a wide range of cytoplasmic signaling proteins. The recent cloning of three human filamins and studies on filamin orthologues from chicken and Drosophila revealed unexpected complexity of the filamin family, the biological implications of which have just started to be addressed. Expression of dysfunctional filamin-A leads to the genetic disorder of ventricular heterotopia and gives reason to expect that abnormalities in the other isogenes may also be connected with human disease. In this review aspects of filamin structure, its splice variants, binding partners and biological function will be discussed.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Actins / metabolism
  • Amino Acid Sequence
  • Animals
  • Binding Sites
  • Contractile Proteins / chemistry
  • Contractile Proteins / genetics
  • Contractile Proteins / metabolism*
  • Filamins
  • Humans
  • Membrane Proteins / metabolism
  • Microfilament Proteins / chemistry
  • Microfilament Proteins / genetics
  • Microfilament Proteins / metabolism*
  • Models, Molecular
  • Molecular Sequence Data
  • Molecular Structure
  • Mutation
  • Sequence Alignment
  • Signal Transduction
  • Species Specificity

Substances

  • Actins
  • Contractile Proteins
  • Filamins
  • Membrane Proteins
  • Microfilament Proteins