Lack of interaction between between NEMO and SHARPIN impairs linear ubiquitination and NF-κB activation, and leads to incontinentia pigmenti

We identified for the first time in human the NEMO domain involved in SHARPIN interaction which is dispensable for NF-κB signaling. Beside, HOIL-1, HOIP, and OTULIN deficiency we confirmed that defect of linear ubiquitination is responsible for human diseases.

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