A structural scaffolding of intermediate filaments in health and disease.
The cytoplasm of animal cells is structured by a scaffolding composed of actin microfilaments, microtubules, and intermediate filaments. Intermediate filaments, so named because their 10-nanometer diameter is intermediate between that of microfilaments (6 nanometers) and microtubules (23 nanometers), assemble into an anastomosed network within the cytoplasm. In combination with a recently identified class of cross-linking proteins that mediate interactions between intermediate filaments and the other cytoskeletal networks, evidence is reviewed here that intermediate filaments provide a flexible intracellular scaffolding whose function is to structure cytoplasm and to resist stresses externally applied to the cell. Mutations that weaken this structural framework increase the risk of cell rupture and cause a variety of human disorders.
Fuchs
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Howard Hughes Medical Institute and Department of Molecular Genetics and Cell Biology, University of Chicago, Chicago, IL 60637, USA.
Cleveland
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DW
D000818:Animals; D001369:Axons / Q000502:physiology / Q000648:ultrastructure; D003593:Cytoplasm / Q000502:physiology / Q000648:ultrastructure; D003598:Cytoskeletal Proteins / Q000378:metabolism*; D004194:Disease; D030342:Genetic Diseases, Inborn / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007381:Intermediate Filament Proteins / Q000378:metabolism*; D007382:Intermediate Filaments / Q000502:physiology* / Q000648:ultrastructure; D015603:Keratinocytes / Q000502:physiology / Q000648:ultrastructure; D007633:Keratins / Q000235:genetics / Q000502:physiology; D008870:Microtubules / Q000378:metabolism / Q000648:ultrastructure; D009154:Mutation; D009474:Neurons / Q000502:physiology / Q000648:ultrastructure
D003598:Cytoskeletal Proteins; D007381:Intermediate Filament Proteins; D007633:Keratins