Recent developments of the investigations on the molecular basis of anhidrotic ectodermal dysplasia are reviewed. Identification of the major product of the EDA gene (ectodysplasin A), a protein belonging to a group of TNF ligands, and molecular cloning of the cDNA, encoding its receptor (EDAR), a member of the TNF receptor family, are presented. The role of an alternative EDA receptor, localised on the X chromosome (XEDAR) in the developmental control of the differentiation of skin appendages, is discussed. Recent findings have elucidated the cause of the autosomal forms of EDA, both dominant and recessive, and indicated an important role of a signal transduction pathway involving a protein product of the NEMO gene and the transcription factor NFkB in the differentiation of skin appendages.
Fas ligand (FasL) induces apoptosis of cells, including activated lymphocytes, expressing its cognate receptor, Fas (CD95/APO-1). FasL precludes inflammatory reactions from immune privileged sites by triggering Fas-mediated apoptosis of infiltrating proinflammatory cells. Aberrant expression of FasL by cancers inhibits antitumor immune responses. The ability of FasL to impair immune responses may hold therapeutic promise as a means of protecting tissue transplants from immunological rejection. Paradoxically, FasL exhibits proinflammatory activity independent of its ability to mediate immune privilege. FasL has been shown to recruit and activate neutrophils, although the factors that determine whether FasL is pro- or anti-inflammatory are only beginning to emerge. FasL appears to contribute to cell death in Fas-sensitive endorgan cells during inflammation. Blocking of Fas-mediated endorgan apoptosis or enhancing Fas-mediated apoptosis of inflammatory cells represent potential targets for future antiinflammatory therapies.
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