Intermediate filament assembly: dynamics to disease

Trends Cell Biol. 2008 Jan;18(1):28-37. doi: 10.1016/j.tcb.2007.11.004.

Abstract

Intermediate filament (IF) proteins belong to a large and diverse gene family with broad representation in vertebrate tissues. Although considered the 'toughest' cytoskeletal fibers, studies in cultured cells have revealed that IF can be surprisingly dynamic and highly regulated. This review examines the diversity of IF assembly behaviors, and considers the ideas that IF proteins are co- or post-translationally assembled into oligomeric precursors, which can be delivered to different subcellular compartments by microtubules or actomyosin and associated motor proteins. Their interaction with other cellular elements via IF associated proteins (IFAPs) affects IF dynamics and also results in cellular networks with properties that transcend those of individual components. We end by discussing how mutations leading to defects in IF assembly, network formation or IF-IFAP association compromise in vivo functions of IF as protectors against environmental stress.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cardiomyopathies / etiology*
  • Cell Line
  • Charcot-Marie-Tooth Disease / etiology*
  • Cytoplasm
  • Humans
  • Intermediate Filament Proteins / genetics*
  • Intermediate Filament Proteins / metabolism
  • Intermediate Filaments / metabolism*
  • Mice
  • Muscular Dystrophies / etiology*
  • Mutation*
  • Rats

Substances

  • Intermediate Filament Proteins