Filamins in cell signaling, transcription and organ development.
Filamins are large actin-binding proteins that stabilize delicate three-dimensional actin filament networks and link them to cellular membranes where they integrate cell architectural and signaling functions important for cell locomotion. Filamins have been shown to bind to proteins with diverse functions and are implicated in human genetic diseases including malformations of the skeleton, brain, and heart. Mouse models of filamin deficiency have advanced our understanding of the important roles filamins play in embryonic development and disease progression. These studies provide clear evidence that cytoskeletal filamin proteins integrate cell signaling, transcription and organ development. This review focuses on the emerging roles of filamins in cell signaling and transcription, with emphasis on cell motility and organ development.
Zhou
Alex-Xianghua
AX
Department of Medical Biochemistry and Cell Biology, Institute of Biomedicine, University of Gothenburg, SE-405 30 Göteborg, Sweden.
Hartwig
John H
JH
Akyürek
Levent M
LM
D000818:Animals; D002465:Cell Movement / Q000502:physiology*; D000013:Congenital Abnormalities / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D003285:Contractile Proteins / Q000235:genetics / Q000378:metabolism*; D003599:Cytoskeleton / Q000235:genetics / Q000378:metabolism* / Q000648:ultrastructure; D004195:Disease Models, Animal; D064448:Filamins; D006801:Humans; D051379:Mice; D008840:Microfilament Proteins / Q000235:genetics / Q000378:metabolism*; D020559:Monomeric GTP-Binding Proteins / Q000235:genetics / Q000378:metabolism; D038081:Organogenesis / Q000502:physiology*; D050436:Regulatory Elements, Transcriptional / Q000502:physiology; D015398:Signal Transduction / Q000502:physiology*; D015533:Transcriptional Activation / Q000502:physiology
D003285:Contractile Proteins; D064448:Filamins; D008840:Microfilament Proteins; D020559:Monomeric GTP-Binding Proteins