Intermediate Filaments 2008

Intermediate filament (IF) proteins are encoded by one of the largest gene families in man. They have attracted considerable attention because mutations in IF genes cause, according to their tissue-specific expression, a large variety of severe human d...

Intermediate filament (IF) proteins are encoded by one of the largest gene families in man. They have attracted considerable attention because mutations in IF genes cause, according to their tissue-specific expression, a large variety of severe human diseases. Most notably, mutations affecting the nuclear intermediate filament proteins lamin A and C lead to a stunning multitude of disease entities ranging from muscular dystrophies to premature ageing. In order to obtain more insight into IF-dependent cellular functions and disease entities, various cell culture systems and animal models, in particular transgenic mice, have been generated. Complementary to this field-wide “bench-to-bed” enterprise an arsenal of biophysical techniques is being applied to IFs aimed at dissecting the structure, assembly and mechanics of single filaments at atomic detail.

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