Hereditary skin diseases of hemidesmosomes

Studies of hereditary blistering skin diseases (epidermolysis bullosa) and targeted gene mutation experiments in knockout mice have greatly improved our understanding of hemidesmosomes and their associated structures in the cytoskeleton and basement membrane of the skin and mucous membranes. At least 10 molecules are recruited in hemidesmosome complexes, where they interact in a complex way. Hemidesmosomes are not simple adhesion devices, but also transduce signals for cell spreading, cell proliferation and basement membrane organisation. The dynamics of a hemidesmosome raises the metaphor of a self-assembling suspension bridge which evokes activities on both sides of the river. This review summarises our current knowledge of the molecular pathology of hemidesmosomes caused by hereditary skin disease or gene targeting experiment.