Hemidesmosomes and their unique transmembrane protein BP180

Hemidesmosomes are adhesion complexes responsible for linking keratin intermediate filaments of stratified and complex epithelia to components of the extracellular matrix such as collagen fibrils. Over the past several years, it has become clear that there are at least five hemidesmosomal proteins, including HD1/plectin and BP230 as cytoplasmic plaque proteins and integrin alpha6beta4 and BP180 as transmembrane proteins. Among them, BP180 is unique as a transmembrane protein because of its collagenous extracellular domain. Recent biochemical and ultrastructural analyses have revealed its molecular configuration and nature as a major component of anchoring filaments connecting hemidesmosomes to the basement membrane. These results indicate that BP180 is a new type of adhesion receptor. In addition to biochemical analyses of these hemidesmosomal proteins, recent studies on patients with inherited skin blistering diseases and on knockout mice have demonstrated roles in hemidesmosome formation and stabilization, as well as unexpected, novel functions.