Desmosomal cell adhesion in mammalian development

Abstract

Defects in desmosome-mediated cell-cell adhesion can lead to tissue fragility syndromes. Both inherited and acquired diseases caused by desmosomal defects have been described. The two organs that appear most vulnerable to these defects are the skin with its appendages, and the heart. Furthermore, the analysis of genetically engineered mice has led to the discovery that desmosomal proteins are also required for normal embryonic development. Knockout mice for several desmosomal proteins die in utero. Depending on the protein studied, death occurs either around the time of implantation, at mid-gestation or shortly before birth. So far, it appears that structural defects leading to abnormal histo-architecture and tissue fragility are the main cause of death, i.e. there is no evidence that loss of a desmosomal protein would abort specific cell lineages or differentiation programs. Nevertheless, we are only beginning to understand the functions of individual desmosomal proteins during development. This review focuses on the role of desmosomes during mouse embryonic development.