Close = well-designed? Seems not so...

The T-cell- dependent monogenic primary immunodeficiency diseases are the best understood at the genetic level, in part due to close experimental models in mice resulting from inbreeding or gene knockout. The classic examples are autoimmune lymphoproliferative syndrome (ALPS) and autoimmune polyendocrinopathy-candidiasis-ectodermal dysplasia (APECED, also called autoimmune polyendocrine syndrome type 1 (APS-1)). Together with these is usually included the syndrome of immune dysregulation, polyendocrinopathy, enteropathy, x-linked (IPEX), notwithstanding this in fact being a sex-linked immunodeficiency disorder. Each shows a high incidence of autoimmune features (Table 1).