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EDI200 is a recombinant fusion protein consisting of the human immunoglobulin G1 (IgG1) Fc region fused to the receptor-binding domain of ectodysplasin-A1 (EDA-A1). It is designed as an EDA-A1 replacement therapy for X-linked hypohidrotic ectodermal dysplasia (XLHED), a rare genetic disorder caused by mutations in the EDA gene leading to deficient or absent functional ectodysplasin. By binding specifically to the ectodysplasin A receptor, EDI200 activates signaling pathways necessary for normal development of skin appendages, sweat glands, teeth, and other ectodermal structures. Preclinical studies in animal models demonstrated that postnatal administration can correct key features of XLHED. The drug has been evaluated in clinical trials for safety and efficacy in neonates and adults with XLHED[1][2][5][6].
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