Information for Patients – What is EDI200?

Ectodysplasin – A1 (EDA-A1) is a protein that occurs naturally in healthy people where it is involved in the formation and development of skin and teeth – this protein is missing in patients with XLHED. EDI200 is a form of EDA-A1 being developed by Edimer as a treatment for certain patients with X-linked Hypohidrotic Ectodermal Dysplasia (XLHED). Click through the slides below to see a depiction of the theory behind EDI200.

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The laboratories of Prof. J. Tschopp and Prof. P. Schneider in Lausanne, Switzerland developed a synthetic version of EDA-A1 that has shown promising results in animals with a disease similar to XLHED. This research has lead to the development of EDI200. The rationale for the use of EDI200 in XLHED patients is based on the lack of functional EDA-A1 protein in these patients – which affects the formation of ectodermal structures (including skin and teeth). EDI200 has been shown to substitute for this protein during development in mice and dogs (see Figure 1).

Scientific Rationale

Scientific Rationale

In a particular species of mouse (known as Tabby), EDA-A1 is missing and they have many of the symptoms associated with XLHED. Treatment of pregnant Tabby mice with EDI200 permanently corrected the appearance of the offspring. Equivalent results were achieved after injecting the protein into Tabby mice shortly after birth. In addition dogs lacking EDA-A1(and with an appearance quite similar to humans with XLHED) have been treated successfully with EDI200.