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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease hypereosinophilic syndrome
Comorbidity C0011603|dermatitis
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PubMedID- 20811394 Here, we demonstrate that loss of function of shank-associated rh domain interacting protein (sharpin) leads to activation of nf-kappab signaling in skin, resulting in the development of an idiopathic hypereosinophilic syndrome (ihes) with eosinophilic dermatitis in c57bl/kalawrij-sharpin(cpdm)/rijsunj mice, and clonal expansion of b-1 b cells and cd3(+)cd4(-)cd8(-) t cells.

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