Article
Epidermolysis bullosa simplex-type mutations alter the dynamics of the keratin cytoskeleton and reveal a contribution of actin to the transport of keratin subunits.
Molecular biology of the cell - 1 Mar 2004
Werner Nicola Susann, Windoffer Reinhard, Strnad Pavel, Grund Christine, Leube Rudolf Eberhard, Magin Thomas Michael
Abstract excerpt
Dominant keratin mutations cause epidermolysis bullosa simplex by transforming keratin (K) filaments into aggregates. As a first step toward understanding the properties of mutant keratins in vivo, we stably transfected epithelial cells with an enhanced yellow fluorescent protein-tagged K14R125C mutant. K14R125C became localized as aggregates in the cell periphery and incorporated into perinuclear keratin...
Topics
- Actins
- Biological Transport
- Cells, Cultured
- Cytoskeleton
- Epidermolysis Bullosa Simplex
- Humans
- Keratinocytes
- Keratins
- Mutation
- RNA, Small Interfering
- Recombinant Proteins