Intermediate Filaments and Diseases

Abstract: Intermediate filaments (IFs) together with microtubules and microfilaments form the cytoskeleton network. IFs represent a large family of proteins comprising 73 members， which form the most complex system among three cytoskeletons. In addition to scaffolding function， IFs form complicated signaling platforms and interact with various kinases， receptors， and apoptotic proteins. The number of IF-related diseases is still growing， at least 80 distinct human diseases， including skin blistering， muscular dystrophy， cardiomyopathy， premature aging syndromes， neurodegenerative disorders， and cataract. Furthermore， IF-containing inclusion bodies are characteristic features of several neurodegenerative， muscular， and other disorders. IFs can be used as tags of specific cell types and IF antibodies are widely used in pathological diagnosis. This review introduces the progress of IF-related diseases， especially the mechanisms， which will enhance the readers to understand the role of IFs.

CSTR: 32200.14.cjcb.2011.09.0016