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. 2015 Jun 1;212(6):939–951. doi: 10.1084/jem.20141130

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© 2015 Boisson et al.

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Figure 2. — HOIP deficiency. (A) Relative HOIP mRNA levels in SV40-immortalized fibroblasts from controls, the HOIP-deficient patient (Patient), and HOIL-1–deficient patients (two independent experiments). (B) Expression of LUBAC subunits in control, HOIP-, and HOIL-1–deficient fibroblasts (left) and control and HOIP-deficient EBV-B cells (right) by immunoblotting total cell extracts with the indicated antibodies (two independent experiments). (C) Immunoblot of immunoprecipitated extracts from control, HOIP-, and HOIL-1–deficient fibroblasts (left) and control and HOIP-deficient EBV-B cells (right) with antibodies directed against HOIP (IP-HOIP) or SHARPIN (IP-SHARPIN) and detected with HOIL-1, HOIP, or SHARPIN antibodies (two independent experiments).